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Journal articles on the topic 'Wobbler mice'

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Published: 9 March 2023

Last updated: 10 March 2023

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1

Vacca-Galloway, L., Y. Q. Zhang, P. Bose, and S. H. Zhang. "Alterations in the number of motoneurons containing immunoreactive calcitonin gene-related peptide(CGRP)& choline acetyltransferase(ChAT) in the cervical spinal cord of the wobbler mouse during the development of the motoneuron disease." Proceedings, annual meeting, Electron Microscopy Society of America 53 (August 13, 1995): 970–71. http://dx.doi.org/10.1017/s0424820100141226.

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The Wobbler mouse (wr) has been studied as a model for inherited human motoneuron diseases (MNDs). Using behavioral tests for forelimb power, walking, climbing, and the “clasp-like reflex” response, the progress of the MND can be categorized into early (Stage 1, age 21 days) and late (Stage 4, age 3 months) stages. Age-and sex-matched normal phenotype littermates (NFR/wr) were used as controls (Stage 0), as well as mice from two related wild-type mouse strains: NFR/N and a C57BI/6N. Using behavioral tests, we also detected pre-symptomatic Wobblers at postnatal ages 7 and 14 days. The mice were anesthetized and perfusion-fixed for immunocytochemical (ICC) of CGRP and ChAT in the spinal cord (C3 to C5).Using computerized morphomety (Vidas, Zeiss), the numbers of IR-CGRP labelled motoneurons were significantly lower in 14 day old Wobbler specimens compared with the controls (Fig. 1). The same trend was observed at 21 days (Stage 1) and 3 months (Stage 4). The IR-CGRP-containing motoneurons in the Wobbler specimens declined progressively with age.

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2

Petit, Constance S., Jane J. Lee, Sebastian Boland, Sharan Swarup, Romain Christiano, Zon Weng Lai, Niklas Mejhert, et al. "Inhibition of sphingolipid synthesis improves outcomes and survival in GARP mutant wobbler mice, a model of motor neuron degeneration." Proceedings of the National Academy of Sciences 117, no. 19 (April 28, 2020): 10565–74. http://dx.doi.org/10.1073/pnas.1913956117.

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Numerous mutations that impair retrograde membrane trafficking between endosomes and the Golgi apparatus lead to neurodegenerative diseases. For example, mutations in the endosomal retromer complex are implicated in Alzheimer’s and Parkinson’s diseases, and mutations of the Golgi-associated retrograde protein (GARP) complex cause progressive cerebello-cerebral atrophy type 2 (PCCA2). However, how these mutations cause neurodegeneration is unknown. GARP mutations in yeast, including one causing PCCA2, result in sphingolipid abnormalities and impaired cell growth that are corrected by treatment with myriocin, a sphingolipid synthesis inhibitor, suggesting that alterations in sphingolipid metabolism contribute to cell dysfunction and death. Here we tested this hypothesis in wobbler mice, a murine model with a homozygous partial loss-of-function mutation in Vps54 (GARP protein) that causes motor neuron disease. Cytotoxic sphingoid long-chain bases accumulated in embryonic fibroblasts and spinal cords from wobbler mice. Remarkably, chronic treatment of wobbler mice with myriocin markedly improved their wellness scores, grip strength, neuropathology, and survival. Proteomic analyses of wobbler fibroblasts revealed extensive missorting of lysosomal proteins, including sphingolipid catabolism enzymes, to the Golgi compartment, which may contribute to the sphingolipid abnormalities. Our findings establish that altered sphingolipid metabolism due to GARP mutations contributes to neurodegeneration and suggest that inhibiting sphingolipid synthesis might provide a useful strategy for treating these disorders.

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3

Klatt, Theis, Hahn, Theiss, and Matschke. "Deregulated miR-29b-3p Correlates with Tissue-Specific Activation of Intrinsic Apoptosis in An Animal Model of Amyotrophic Lateral Sclerosis." Cells 8, no. 9 (September 12, 2019): 1077. http://dx.doi.org/10.3390/cells8091077.

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Amyotrophic lateral sclerosis (ALS) is one of the most common incurable motor neuron disorders in adults. The majority of all ALS cases occur sporadically (sALS). Symptoms of ALS are caused by a progressive degeneration of motor neurons located in the motor cortex and spinal cord. The question arises why motor neurons selectively degenerate in ALS, while other cells and systems appear to be spared the disease. Members of the intrinsic apoptotic pathway are frequent targets of altered microRNA expression. Therefore, microRNAs and their effects on cell survival are subject of controversial debates. In this study, we investigated the expression of numerous members of the intrinsic apoptotic cascade by qPCR, western blot, and immunostaining in two different regions of the CNS of wobbler mice. Further we addressed the expression of miR-29b-3p targeting BMF, Bax, and, Bak, members of the apoptotic pathway. We show a tissue-specific differential expression of BMF, Bax, and cleaved-Caspase 3 in wobbler mice. An opposing regulation of miR-29b-3p expression in the cerebellum and cervical spinal cord of wobbler mice suggests different mechanisms regulating the intrinsic apoptotic pathway. Based on our findings, it could be speculated that miR-29b-3p might regulate antiapoptotic survival mechanisms in CNS areas that are not affected by neurodegeneration in the wobbler mouse ALS model.

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4

Ochiai, Akira, Rie Igarashi, Mitsuko Takenaga, Jiro Hoshino, Ken Ikeda, Sadatomo Shimojo, and Yutaka Mizushima. "SOD activity, NOx, SH level in wobbler mice." Ensho 20, no. 1 (1999): 57–63. http://dx.doi.org/10.2492/jsir1981.20.57.

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5

Mitsumoto, Hiroshi, Kozo Kurahashi, Jane M. Jacob, and Irvine G. McQuarrie. "Retardation of fast axonal transport in wobbler mice." Muscle & Nerve 16, no. 5 (May 1993): 542–47. http://dx.doi.org/10.1002/mus.880160517.

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6

Krieger, Charles, Thomas L. Perry, Shirley Hansen, Hiroshi Mitsumoto, and Tage Honoré. "Excitatory Amino Acid Receptor Antagonist in Murine Motoneuron Disease (The Wobbler Mouse)." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 19, no. 4 (November 1992): 462–65. http://dx.doi.org/10.1017/s0317167100041652.

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ABSTRACT:Recent evidence has suggested a potential role for involvement of excitatory amino acids (EAA) in the pathogenesis of the neuron loss in motoneuron diseases. We have examined the ability of an antagonist of N-methyl-D-aspartate (NMDA) receptors to halt or retard the progression of neurological symptoms in a murine form of motoneuron disease. The wobbler mouse is an autosomal recessive mutant which develops progressive neurological symptoms secondary to motoneuron loss. Treatment of wobbler mice with the NMDA receptor antagonist (+)-5-methyl-10,l 1-dihydro-5H-dibenzo(a,d)cyclohepten-5, 10-imine maleate (MK-801) did not retard neurological deterioration as assessed by a semiquantitive clinical scale. We conclude that NMDA receptor activation is probably not involved in the pathogenesis of motoneuron loss in the wobbler mouse.

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7

Iwamoto, Konosuke, Yasuhiro Yoshii, and Ken Ikeda. "Atorvastatin treatment attenuates motor neuron degeneration in wobbler mice." Amyotrophic Lateral Sclerosis 10, no. 5-6 (January 2009): 405–9. http://dx.doi.org/10.3109/17482960902870993.

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8

LaVail, Jennifer H., Edward H. Koo, and Nusi P. Dekker. "Motoneuron loss in the abducens nucleus of wobbler mice." Brain Research 404, no. 1-2 (February 1987): 127–32. http://dx.doi.org/10.1016/0006-8993(87)91363-1.

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9

Deniselle, Maria Claudia Gonzalez, Maria Cecilia Carreras, Laura Garay, Gisella Gargiulo-Monachelli, Maria Meyer, Juan Jose Poderoso, and Alejandro F. De Nicola. "Progesterone prevents mitochondrial dysfunction in the spinal cord of wobbler mice." Journal of Neurochemistry 122, no. 1 (June 11, 2012): 185–95. http://dx.doi.org/10.1111/j.1471-4159.2012.07753.x.

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10

La Vail, Jennifer H., and Katherine P. Irons. "Abnormal neuromuscular junctions in the lateral rectus muscle of wobbler mice." Brain Research 463, no. 1 (October 1988): 78–89. http://dx.doi.org/10.1016/0006-8993(88)90529-x.

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11

Corvino, Valentina, Rita Businaro, Maria Concetta Geloso, Paolo Bigini, Valentina Cavallo, Elena Pompili, Tiziana Mennini, Lorenzo Fumagalli, and Fabrizio Michetti. "S100B Protein and 4-Hydroxynonenal in the Spinal Cord of Wobbler Mice." Neurochemical Research 28, no. 2 (February 2003): 341–45. http://dx.doi.org/10.1023/a:1022345720852.

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12

Smith, Margaret E., and Sharon Hughes. "POMC neuropeptides and their receptors in the neuromuscular system of wobbler mice." Journal of the Neurological Sciences 124 (July 1994): 56–58. http://dx.doi.org/10.1016/0022-510x(94)90178-3.

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13

Mitsumoto, H., K. Ikeda, B. Klinkosz, J. Cedarbaum, V. Wong, and R. Lindsay. "Arrest of motor neuron disease in wobbler mice cotreated with CNTF and BDNF." Science 265, no. 5175 (August 19, 1994): 1107–10. http://dx.doi.org/10.1126/science.8066451.

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14

Röderer, Pascal, Lara Klatt, Felix John, Verena Theis, Konstanze F. Winklhofer, Carsten Theiss, and Veronika Matschke. "Increased ROS Level in Spinal Cord of Wobbler Mice due to Nmnat2 Downregulation." Molecular Neurobiology 55, no. 11 (March 16, 2018): 8414–24. http://dx.doi.org/10.1007/s12035-018-0999-7.

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15

Dennis, J. S., and B. A. Citron. "Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein." Neuroscience 158, no. 2 (January 2009): 745–50. http://dx.doi.org/10.1016/j.neuroscience.2008.10.030.

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16

Ukabam, Chinyere U. "A Golgi-Cox study of the hypoglossal nucleus of ‘wobbler’ and normal mice." La Ricerca in Clinica e in Laboratorio 18, no. 4 (October 1988): 313–18. http://dx.doi.org/10.1007/bf02919089.

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17

Zwilling, Mareike, Carsten Theiss, and Veronika Matschke. "Caffeine and NAD+ Improve Motor Neural Integrity of Dissociated Wobbler Cells In Vitro." Antioxidants 9, no. 6 (May 27, 2020): 460. http://dx.doi.org/10.3390/antiox9060460.

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Amyotrophic lateral sclerosis (ALS) is a common degenerative disease of the central nervous system concerning a progressive loss of upper and lower motor neurons. While 5%–10% of patients are diagnosed with the inherited form of the disease, the vast majority of patients suffer from the less characterized sporadic form of ALS (sALS). As the wobbler mouse and the ALS show striking similarities in view of phenotypical attributes, the mouse is rated as an animal model for the disease. Recent investigations show the importance of nicotinamide adenine dinucleotide (NAD+) and its producing enzyme nicotinic acid mononucleotide transferase 2 (Nmnat2) for neurodegeneration as well as for the preservation of health of the neuronal cells. Furthermore, it is newly determined that these molecules show significant downregulations in the spinal cord of wobbler mice in the stable phase of disease development. Here, we were able to prove a positive benefit on affected motor neurons from an additional NAD+ supply as well as an increase in the Nmnat2 level through caffeine treatment in cells in vitro. In addition, first assumptions about the importance of endogenous and exogenous factors that have an influence on the wellbeing of motor nerve cells in the model of ALS can be considered.

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18

Laage, Stephan, Gerald Zobel, and Harold Jockusch. "Astrocyte Overgrowth in the Brain Stem and Spinal Cord of Mice Affected by Spinal Atrophy, Wobbler." Developmental Neuroscience 10, no. 3 (1988): 190–98. http://dx.doi.org/10.1159/000111969.

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19

Meyer, Maria, Agustina Lara, Hazel Hunt, Joseph Belanoff, E. Ronald de Kloet, Maria Claudia Gonzalez Deniselle, and Alejandro F. De Nicola. "The Selective Glucocorticoid Receptor Modulator Cort 113176 Reduces Neurodegeneration and Neuroinflammation in Wobbler Mice Spinal Cord." Neuroscience 384 (August 2018): 384–96. http://dx.doi.org/10.1016/j.neuroscience.2018.05.042.

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20

Meyer, Maria, Maria Claudia Gonzalez Deniselle, Hazel Hunt, E. Ronald de Kloet, and Alejandro F. De Nicola. "The selective glucocorticoid receptor modulator CORT108297 restores faulty hippocampal parameters in Wobbler and corticosterone-treated mice." Journal of Steroid Biochemistry and Molecular Biology 143 (September 2014): 40–48. http://dx.doi.org/10.1016/j.jsbmb.2014.02.007.

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21

Ott, Bastian, Carolin Dahlke, Karl Meller, Markus Napirei, Thomas Schmitt-John, Beate Brand-Saberi, Carsten Theiss, and Darius Saberi. "Implementation of a manual for working with wobbler mice and criteria for discontinuation of the experiment." Annals of Anatomy - Anatomischer Anzeiger 200 (July 2015): 118–24. http://dx.doi.org/10.1016/j.aanat.2015.03.007.

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22

Dave, K. R., R. Prado, R. Busto, A. P. Raval, W. G. Bradley, D. Torbati, and M. A. Pérez-pinzón. "Hyperbaric oxygen therapy protects against mitochondrial dysfunction and delays onset of motor neuron disease in wobbler mice." Neuroscience 120, no. 1 (August 2003): 113–20. http://dx.doi.org/10.1016/s0306-4522(03)00244-6.

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23

Diana, Valentina, Arianna Ottolina, Francesca Botti, Elena Fumagalli, Eleonora Calcagno, Massimiliano De Paola, Alfredo Cagnotto, et al. "Neural precursor-derived astrocytes of wobbler mice induce apoptotic death of motor neurons through reduced glutamate uptake." Experimental Neurology 225, no. 1 (September 2010): 163–72. http://dx.doi.org/10.1016/j.expneurol.2010.06.008.

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24

Bose, P., R. Fielding, K. M. Ameis, and L. L. Vacca-Galloway. "A novel behavioral method to detect motoneuron disease in Wobbler mice aged three to seven days old." Brain Research 813, no. 2 (December 1998): 334–42. http://dx.doi.org/10.1016/s0006-8993(98)01033-6.

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25

Ono, Satoshi, and Hirokazu Narita. "Development of a novel cognition enhancer, T-588. –Its protective effects on cultured cells and neurodegenerative Wobbler mice–." Japanese Journal of Pharmacology 82 (2000): 29. http://dx.doi.org/10.1016/s0021-5198(19)47595-1.

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26

Paiardi, Chiara, Maria Enrica Pasini, Alida Amadeo, Mariarosa Gioria, and Giovanna Berruti. "The ESCRT-deubiquitinating enzyme USP8 in the cervical spinal cord of wild-type and Vps54-recessive (wobbler) mutant mice." Histochemistry and Cell Biology 141, no. 1 (April 25, 2013): 57–73. http://dx.doi.org/10.1007/s00418-013-1096-7.

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27

Festoff, Barry W., Michael R. D’Andrea, Bruce A. Citron, Raymundo M. Salcedo, Irina V. Smirnova, and Patricia Andrade-Gordon. "Motor Neuron Cell Death in Wobbler Mutant Mice Follows Overexpression of the G-protein-coupled, Protease-activated Receptor for Thrombin." Molecular Medicine 6, no. 5 (May 2000): 410–29. http://dx.doi.org/10.1007/bf03401784.

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28

Pérez-Victoria, F. Javier, Gonzalo A. Mardones, and Juan S. Bonifacino. "Requirement of the Human GARP Complex for Mannose 6-phosphate-receptor-dependent Sorting of Cathepsin D to Lysosomes." Molecular Biology of the Cell 19, no. 6 (June 2008): 2350–62. http://dx.doi.org/10.1091/mbc.e07-11-1189.

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The biosynthetic sorting of acid hydrolases to lysosomes relies on transmembrane, mannose 6-phosphate receptors (MPRs) that cycle between the TGN and endosomes. Herein we report that maintenance of this cycling requires the function of the mammalian Golgi-associated retrograde protein (GARP) complex. Depletion of any of the three GARP subunits, Vps52, Vps53, or Vps54, by RNAi impairs sorting of the precursor of the acid hydrolase, cathepsin D, to lysosomes and leads to its secretion into the culture medium. As a consequence, lysosomes become swollen, likely due to a buildup of undegraded materials. Missorting of cathepsin D in GARP-depleted cells results from accumulation of recycling MPRs in a population of light, small vesicles downstream of endosomes. These vesicles might correspond to intermediates in retrograde transport from endosomes to the TGN. Depletion of GARP subunits also blocks the retrograde transport of the TGN protein, TGN46, and the B subunit of Shiga toxin. These observations indicate that the mammalian GARP complex plays a general role in the delivery of retrograde cargo into the TGN. We also report that a Vps54 mutant protein in the Wobbler mouse strain is active in retrograde transport, thus explaining the viability of these mutant mice.

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29

Deniselle, Maria Claudia Gonzalez, Susana L. Gonzalez, Gerardo G. Piroli, Analia E. Lima, and Alejandro F. De Nicola. "The 21-aminosteroid U-74389F increases the number of glial fibrillary acidic protein-expressing astrocytes in the spinal cord of control and wobbler mice." Cellular and Molecular Neurobiology 16, no. 1 (February 1996): 61–72. http://dx.doi.org/10.1007/bf02578387.

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30

Court, J. A., J. R. McDermott, A. M. Gibson, E. Marshall, C. A. Bloxham, R. H. Perry, and J. A. Edwardson. "Raised Thyrotrophin-Releasing Hormone, Pyroglutamylamino Peptidase, and Proline Endopeptidase Are Present in the Spinal Cord of Wobbler Mice but Not in Human Motor Neurone Disease." Journal of Neurochemistry 49, no. 4 (October 1987): 1084–90. http://dx.doi.org/10.1111/j.1471-4159.1987.tb09997.x.

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31

Songe-Møller, Lene, Erwin van den Born, Vibeke Leihne, Cathrine B. Vågbø, Terese Kristoffersen, Hans E. Krokan, Finn Kirpekar, Pål Ø. Falnes, and Arne Klungland. "Mammalian ALKBH8 Possesses tRNA Methyltransferase Activity Required for the Biogenesis of Multiple Wobble Uridine Modifications Implicated in Translational Decoding." Molecular and Cellular Biology 30, no. 7 (February 1, 2010): 1814–27. http://dx.doi.org/10.1128/mcb.01602-09.

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ABSTRACT Uridines in the wobble position of tRNA are almost invariably modified. Modifications can increase the efficiency of codon reading, but they also prevent mistranslation by limiting wobbling. In mammals, several tRNAs have 5-methoxycarbonylmethyluridine (mcm5U) or derivatives thereof in the wobble position. Through analysis of tRNA from Alkbh8 −/− mice, we show here that ALKBH8 is a tRNA methyltransferase required for the final step in the biogenesis of mcm5U. We also demonstrate that the interaction of ALKBH8 with a small accessory protein, TRM112, is required to form a functional tRNA methyltransferase. Furthermore, prior ALKBH8-mediated methylation is a prerequisite for the thiolation and 2′-O-ribose methylation that form 5-methoxycarbonylmethyl-2-thiouridine (mcm5s2U) and 5-methoxycarbonylmethyl-2′-O-methyluridine (mcm5Um), respectively. Despite the complete loss of all of these uridine modifications, Alkbh8 −/− mice appear normal. However, the selenocysteine-specific tRNA (tRNASec) is aberrantly modified in the Alkbh8 −/− mice, and for the selenoprotein Gpx1, we indeed observed reduced recoding of the UGA stop codon to selenocysteine.

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32

Jasa, Lie, and I. Putu Ardana. "Disain LieYo Hidro Generator Explorasi Energi Arus Lepas Pantai." Majalah Ilmiah Teknologi Elektro 16, no. 3 (January 10, 2018): 39. http://dx.doi.org/10.24843/mite.2017.v16i03p07.

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Sea currents are one of these renewable energy sources that have not been developed, because they cost a lot and the capacity of energy produced is small. The presence of corrosion factors of sea air to the material containing the iron element of the material used must be of stainless steel. The existence of coral reefs that grow on objects submerged in the sea in a long time. Based on the above problems, the researcher wanted to develop an idea to make LieYo hydro generator. The idea of generating this generator is based on a magnetic ball placed on a vertically mounted pipe and each pipe is paired with a coil, a magnet ball that moves freely up and down in the pipe due to the wobble of the sea surface. Movement of magnetic spheres up and down causing the coils to receive magnetic field lines, the movement of the magnetic sphere will form an electric current on the coil. Several designs were tried out from the results of this study. Later can be used as the basis of designing a LieYo hydro generator.

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33

Moustafa, Mohamed E., Bradley A. Carlson, Muhammad A. El-Saadani, Gregory V. Kryukov, Qi-An Sun, John W. Harney, Kristina E. Hill, et al. "Selective Inhibition of Selenocysteine tRNA Maturation and Selenoprotein Synthesis in Transgenic Mice Expressing Isopentenyladenosine-Deficient Selenocysteine tRNA." Molecular and Cellular Biology 21, no. 11 (June 1, 2001): 3840–52. http://dx.doi.org/10.1128/mcb.21.11.3840-3852.2001.

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ABSTRACT Selenocysteine (Sec) tRNA (tRNA[Ser]Sec) serves as both the site of Sec biosynthesis and the adapter molecule for donation of this amino acid to protein. The consequences on selenoprotein biosynthesis of overexpressing either the wild type or a mutant tRNA[Ser]Sec lacking the modified base, isopentenyladenosine, in its anticodon loop were examined by introducing multiple copies of the corresponding tRNA[Ser]Sec genes into the mouse genome. Overexpression of wild-type tRNA[Ser]Sec did not affect selenoprotein synthesis. In contrast, the levels of numerous selenoproteins decreased in mice expressing isopentenyladenosine-deficient (i6A−) tRNA[Ser]Sec in a protein- and tissue-specific manner. Cytosolic glutathione peroxidase and mitochondrial thioredoxin reductase 3 were the most and least affected selenoproteins, while selenoprotein expression was most and least affected in the liver and testes, respectively. The defect in selenoprotein expression occurred at translation, since selenoprotein mRNA levels were largely unaffected. Analysis of the tRNA[Ser]Sec population showed that expression of i6A− tRNA[Ser]Sec altered the distribution of the two major isoforms, whereby the maturation of tRNA[Ser]Sec by methylation of the nucleoside in the wobble position was repressed. The data suggest that the levels of i6A− tRNA[Ser]Sec and wild-type tRNA[Ser]Sec are regulated independently and that the amount of wild-type tRNA[Ser]Sec is determined, at least in part, by a feedback mechanism governed by the level of the tRNA[Ser]Sec population. This study marks the first example of transgenic mice engineered to contain functional tRNA transgenes and suggests that i6A−tRNA[Ser]Sec transgenic mice will be useful in assessing the biological roles of selenoproteins.

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34

Madarame, H., K. Ogihara, M. Kimura, M. Nagai, T. Omatsu, H. Ochiai, and T. Mizutani. "Detection of pneumonia virus of mice in an african hedgehog (aterelix arbiventris) with suspected ‘wobbly hedgehog syndrome’." Journal of Comparative Pathology 152, no. 1 (January 2015): 88–89. http://dx.doi.org/10.1016/j.jcpa.2014.10.192.

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35

Xu, Fu, Anders S. Byström, and Marcus J. O. Johansson. "SSD1 modifies phenotypes of Elongator mutants." Current Genetics 66, no. 3 (November 27, 2019): 481–85. http://dx.doi.org/10.1007/s00294-019-01048-9.

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AbstractThe translational decoding properties of tRNAs are influenced by post-transcriptional modification of nucleosides in their anticodon region. The Elongator complex promotes the first step in the formation of 5-methoxycarbonylmethyl (mcm5), 5-methoxycarbonylhydroxymethyl (mchm5), and 5-carbamoylmethyl (ncm5) groups on wobble uridine residues in eukaryotic cytosolic tRNAs. Elongator mutants in yeast, worms, plants, mice, and humans not only show a tRNA modification defect, but also a diverse range of additional phenotypes. Even though the phenotypes are almost certainly caused by the reduced functionality of the hypomodified tRNAs in translation, the basis for specific phenotypes is not well understood. Here, we discuss the recent finding that the phenotypes of Saccharomyces cerevisiae Elongator mutants are modulated by the genetic background. This background-effect is largely due to the allelic variation at the SSD1 locus, which encodes an mRNA-binding protein involved in post-transcriptional regulation of gene expression. A nonsense ssd1 allele is found in several wild-type laboratory strains and the presence of this allele aggravates the stress-induced phenotypes of Elongator mutants. Moreover, other phenotypes, such as the histone acetylation and telomeric gene silencing defects, are dependent on the mutant ssd1 allele. Thus, SSD1 is a genetic modifier of the phenotypes of Elongator-deficient yeast cells.

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36

Madarame, Hiroo, Kikumi Ogihara, Moe Kimura, Makoto Nagai, Tsutomu Omatsu, Hideharu Ochiai, and Tetsyuya Mizutani. "Detection of a pneumonia virus of mice (PVM) in an African hedgehog (Atelerix arbiventris) with suspected wobbly hedgehog syndrome (WHS)." Veterinary Microbiology 173, no. 1-2 (September 2014): 136–40. http://dx.doi.org/10.1016/j.vetmic.2014.07.012.

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37

Zhang, Jilei, Rong Lu, Yongguo Zhang, Żaneta Matuszek, Wen Zhang, Yinglin Xia, Tao Pan, and Jun Sun. "tRNA Queuosine Modification Enzyme Modulates the Growth and Microbiome Recruitment to Breast Tumors." Cancers 12, no. 3 (March 9, 2020): 628. http://dx.doi.org/10.3390/cancers12030628.

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Background: Transfer RNA (tRNA) queuosine (Q)-modifications occur specifically in 4 cellular tRNAs at the wobble anticodon position. tRNA Q-modification in human cells depends on the gut microbiome because the microbiome product queuine is required for its installation by the enzyme Q tRNA ribosyltransferase catalytic subunit 1 (QTRT1) encoded in the human genome. Queuine is a micronutrient from diet and microbiome. Although tRNA Q-modification has been studied for a long time regarding its properties in decoding and tRNA fragment generation, how QTRT1 affects tumorigenesis and the microbiome is still poorly understood. Results: We generated single clones of QTRT1-knockout breast cancer MCF7 cells using Double Nickase Plasmid. We also established a QTRT1-knockdown breast MDA-MB-231 cell line. The impacts of QTRT1 deletion or reduction on cell proliferation and migration in vitro were evaluated using cell culture, while the regulations on tumor growth in vivo were evaluated using a xenograft BALB/c nude mouse model. We found that QTRT1 deficiency in human breast cancer cells could change the functions of regulation genes, which are critical in cell proliferation, tight junction formation, and migration in human breast cancer cells in vitro and a breast tumor mouse model in vivo. We identified that several core bacteria, such as Lachnospiraceae, Lactobacillus, and Alistipes, were markedly changed in mice post injection with breast cancer cells. The relative abundance of bacteria in tumors induced from wildtype cells was significantly higher than those of QTRT1 deficiency cells. Conclusions: Our results demonstrate that the QTRT1 gene and tRNA Q-modification altered cell proliferation, junctions, and microbiome in tumors and the intestine, thus playing a critical role in breast cancer development.

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Richard, Patricia, Lucie Kozlowski, Hélène Guillorit, Patrice Garnier, Nicole C. McKnight, Antoine Danchin, and Xavier Manière. "Queuine, a bacterial-derived hypermodified nucleobase, shows protection in in vitro models of neurodegeneration." PLOS ONE 16, no. 8 (August 11, 2021): e0253216. http://dx.doi.org/10.1371/journal.pone.0253216.

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Growing evidence suggests that human gut bacteria, which comprise the microbiome, are linked to several neurodegenerative disorders. An imbalance in the bacterial population in the gut of Parkinson’s disease (PD) and Alzheimer’s disease (AD) patients has been detected in several studies. This dysbiosis very likely decreases or increases microbiome-derived molecules that are protective or detrimental, respectively, to the human body and those changes are communicated to the brain through the so-called ‘gut-brain-axis’. The microbiome-derived molecule queuine is a hypermodified nucleobase enriched in the brain and is exclusively produced by bacteria and salvaged by humans through their gut epithelium. Queuine replaces guanine at the wobble position (position 34) of tRNAs with GUN anticodons and promotes efficient cytoplasmic and mitochondrial mRNA translation. Queuine depletion leads to protein misfolding and activation of the endoplasmic reticulum stress and unfolded protein response pathways in mice and human cells. Protein aggregation and mitochondrial impairment are often associated with neural dysfunction and neurodegeneration. To elucidate whether queuine could facilitate protein folding and prevent aggregation and mitochondrial defects that lead to proteinopathy, we tested the effect of chemically synthesized queuine, STL-101, in several in vitro models of neurodegeneration. After neurons were pretreated with STL-101 we observed a significant decrease in hyperphosphorylated alpha-synuclein, a marker of alpha-synuclein aggregation in a PD model of synucleinopathy, as well as a decrease in tau hyperphosphorylation in an acute and a chronic model of AD. Additionally, an associated increase in neuronal survival was found in cells pretreated with STL-101 in both AD models as well as in a neurotoxic model of PD. Measurement of queuine in the plasma of 180 neurologically healthy individuals suggests that healthy humans maintain protective levels of queuine. Our work has identified a new role for queuine in neuroprotection uncovering a therapeutic potential for STL-101 in neurological disorders.

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Zhang, Jilei, Yong-guo Zhang, Yinglin Xia, and Jun Sun. "TRANSFER RNA QUEUOSINE MODIFICATION ENZYME MANIPULATES TIGHT JUNCTION PROTEINS IN INFLAMMATORY BOWEL DISEASE." Inflammatory Bowel Diseases 27, Supplement_1 (January 1, 2021): S28. http://dx.doi.org/10.1093/ibd/izaa347.065.

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Abstract Background Transfer RNA (tRNA) queuosine (Q)-modification occur at the wobble anticodon position of four special cellular tRNAs. In eukaryotes, tRNA-Q modification relies on the intestinal microbial product queuine and eukaryotic tRNA-guanine transglycosylase complex contains of Q tRNA ribosyltransferase catalytic subunit 1 (QTRT1). Q-tRNA modification is critical for the fidelity and accuracy to translate RNA to protein. It is known that dysfunction of Q-tRNA associates with cancer proliferation and malignancy. However, mechanisms of how Q and Q-tRNA modifications influence intestinal epithelial biology and chronic inflammation are unknown. Methods We explored the expression of QTRT1 in patients with IBD by investigating human biopsy tissues and re-analyzing gene expression datasets (with both UC and CD patients). To further investigate the QTRT1 role in IBD, the dextran sodium sulfate (DSS) induced colitis mouse model and IL10-deficient mouse model were used to evaluate the molecular mechanism. We further investigated the impact of QTRT1 reduction on intestinal cells by generating QTRT1 knockdown HCT116 and Caco-2 BBE cells using a Double Nickase Plasmid. Results We found that the QTRT1 expression in IHC staining was significantly downregulated in the colon of both Ulcerative Colitis (UC) and Crohn’s Disease (CD) patients compared with normal controls. After extracting and analyzing the datasets, we found the gene expression of QTRT1 was significantly suppressed in colonic mucosa of UC and intestinal mucosa of CD, compared with controls. Moreover, the four tRNA-Q-related tRNA synthetase (asparaginyl-tRNA synthetase, aspartyl-tRNA synthetase, histidyl-tRNA synthetase and tyrosyl-tRNA synthetase) were also decreased in IBD patients. The decreased QTRT1-expression was further confirmed in both DSS-induced colitis mice and IL10-defecient mice. Meanwhile, the significantly downregulated beta-catenin and Claudin-5, and upregulated Caludin-2 were identified in these two colitis models. All these molecular alterations, which are important in cell adhesion and tight junction formation, were further verified in protein expression and mRNA level using QTRT1 reduction HCT116 and Caco-2 BBE cells in vitro. Conclusion Our results demonstrate that the QTRT1 plays a novel role in human IBD by altering intestinal cell adhesion and junctions. Microbiome-dependent Q supply can be altered during disease development or when a limited variety of food-types are ingested. Investigations on the gut microbiome-derived tRNA modification in IBD will uncover novel molecular mechanisms for potential prevention and therapy.

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Iwamoto, Konosuke, Yasuhiro Yoshii, and Ken Ikeda. "Atorvastatin treatment attenuates motor neuron degeneration in wobbler mice." Amyotrophic Lateral Sclerosis, 2009, 1–6. http://dx.doi.org/10.1080/17482960902870993.

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Junghans, Maya, Felix John, Hilal Cihankaya, Daniel Schliebs, Konstanze F. Winklhofer, Verian Bader, Johann Matschke, Carsten Theiss, and Veronika Matschke. "ROS scavengers decrease γH2ax spots in motor neuronal nuclei of ALS model mice in vitro." Frontiers in Cellular Neuroscience 16 (August 31, 2022). http://dx.doi.org/10.3389/fncel.2022.963169.

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Background: Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease characterized by the loss of motor neurons in cerebral cortex, brainstem and spinal cord. Numerous studies have demonstrated signs of oxidative stress in postmortem neuronal tissue, cerebrospinal fluid, plasma and urine of ALS patients, without focusing on the specific processes within motor neurons. Thus, we aimed to investigate the relevance of reactive oxygen species (ROS) detoxification mechanisms and its consequences on the formation of toxic/lethal DNA double strand breaks (DSBs) in the ALS model of the Wobbler mouse.Methods: Live cell imaging in dissociated motor neuronal cultures was used to investigate the production of ROS using Dihydroethidium (DHE). The expression levels of ROS detoxifying molecules were investigated by qPCR as well as Western blots. Furthermore, the expression levels of DNA damage response proteins p53bp1 and H2ax were investigated using qPCR and immunofluorescence staining. Proof-of-principle experiments using ROS scavengers were performed in vitro to decipher the influence of ROS on the formation of DNA double strand breaks quantifying the γH2ax spots formation.Results: Here, we verified an elevated ROS-level in spinal motor neurons of symptomatic Wobbler mice in vitro. As a result, an increased number of DNA damage response proteins p53bp1 and γH2ax in dissociated motor neurons of the spinal cord of Wobbler mice was observed. Furthermore, we found a significantly altered expression of several antioxidant molecules in the spinal cord of Wobbler mice, suggesting a deficit in ROS detoxification mechanisms. This hypothesis could be verified by using ROS scavenger molecules in vitro to reduce the number of γH2ax foci in dissociated motor neurons and thus counteract the harmful effects of ROS.Conclusion: Our data indicate that maintenance of redox homeostasis may play a key role in the therapy of the neurodegenerative disease ALS. Our results underline a necessity for multimodal treatment approaches to prolong the average lifespan of motor neurons and thus slow down the progression of the disease, since a focused intervention in one pathomechanism seems to be insufficient in ALS therapy.

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Deniselle, M. C. Gonzalez, L. Garay, M. Meyer, G. Gargiulo-Monachelli, F. Labombarda, S. Gonzalez, R. Guennoun, M. Schumacher, and Alejandro F. De Nicola. "Experimental and clinical evidence for the protective role of progesterone in motoneuron degeneration and neuroinflammation." Hormone Molecular Biology and Clinical Investigation 7, no. 3 (January 1, 2011). http://dx.doi.org/10.1515/hmbci.2011.126.

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AbstractFar beyond its role in reproduction, progesterone exerts neuroprotective, promyelinating, and anti-inflammatory effects in the nervous system. These effects are amplified under pathological conditions, implying that changes of the local environment sensitize nervous tissues to steroid therapy. The present survey covers our results of progesterone neuroprotection in a motoneuron neurodegeneration model and a neuroinflammation model. In the degenerating spinal cord of the Wobbler mouse, progesterone reverses the impaired expression of neurotrophins, increases enzymes of neurotransmission and metabolism, prevents oxidative damage of motoneurons and their vacuolar degeneration (paraptosis), and attenuates the development of mitochondrial abnormalities. After long-term treatment, progesterone also increases muscle strength and the survival of Wobbler mice. Subsequently, this review describes the effects of progesterone in mice with induced experimental autoimmune encephalomyelitis (EAE), a commonly used model of multiple sclerosis. In EAE mice, progesterone attenuates the clinical severity, decreases demyelination and neuronal dysfunction, increases axonal counts, reduces the formation of amyloid precursor protein profiles, and decreases the aberrant expression of growth-associated proteins. These actions of progesterone may be due to multiple mechanisms, considering that classic nuclear receptors, extranuclear receptors, and membrane receptors are all expressed in the spinal cord. Although many aspects of progesterone action in humans remain unsolved, data provided by experimental models makes getting to this objective closer than previously expected.

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Hirayama. "Zonisamide Treatment Delays Motor Neuron Degeneration and Astrocyte Proliferation in Wobbler Mice." Journal of Neurology Research, 2011. http://dx.doi.org/10.4021/jnr59w.

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Bigini, Paolo, Fabrizio Gardoni, Sara Barbera, Alfredo Cagnotto, Elena Fumagalli, Annalisa Longhi, Massimiliano M. Corsi, Monica Di Luca, and Tiziana Mennini. "Expression of AMPA and NMDA receptor subunits in the cervical spinal cord of wobbler mice." BMC Neuroscience 7, no. 1 (October 26, 2006). http://dx.doi.org/10.1186/1471-2202-7-71.

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Narendran, Amithi, Sweta Vangaveti, Srivathsan V. Ranganathan, Emily Eruysal, Miranda Craft, Omar Alrifai, Fu Yee Chua, et al. "Silencing of the tRNA Modification Enzyme Cdkal1 Effects Functional Insulin Synthesis in NIT-1 Cells: tRNALys3 Lacking ms2- (ms2t6A37) is Unable to Establish Sufficient Anticodon:Codon Interactions to Decode the Wobble Codon AAG." Frontiers in Molecular Biosciences 7 (February 9, 2021). http://dx.doi.org/10.3389/fmolb.2020.584228.

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Human Genome Wide Association Studies found a significant risk of Type 2 Diabetes Mellitus (T2DM) in single nucleotide polymorphisms in the cdkal1 gene. The cdkal1 gene is remote from the insulin gene and with the surprising function of a specific tRNA modification. Population studies and case control studies acquired evidences of the connection between Cdkal1 protein and insulin production over the years. To obtain biochemical proofs directly linking potential SNPs to their roles in insulin production and availability is challenging, but the development of Cdkal1 knock out mice and knock out cell lines made it possible to extend our knowledge towards therapeutic field of diabetic research. Supporting the evidences, here we show that knock down of the cdkal1 gene using small interfering and short hairpin RNA in the NIT-1 cell line, a β-cell line inducible for insulin resulted in reduced levels of cdkal1 and mature insulin mRNAs, increased the level of precursor insulin mRNA, decreased Cdkal1 and insulin proteins, and diminished modification of tRNALys3 from t6A37 to ms2t6A37, the specified function of Cdkal1. tRNALys3 lacking ms2- is incapable of establishing sufficient hydrogen bonding energy and hydrophobic stabilization to decode the wobble codon AAG.

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Boada, M., A. Perez-Poch, M. Ballester, M. Tresanchez, E. Sánchez, G. Martínez, D. V. González, S. García, T. Jordi, and N. P. Polyzos. "P-008 Microgravity exposure significantly decreases sperm motility and vitality. Can we consider human reproduction outside the Earth?" Human Reproduction 37, Supplement_1 (June 29, 2022). http://dx.doi.org/10.1093/humrep/deac107.007.

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Abstract Study question Are fresh human sperm samples affected by different gravitational conditions than on Earth? Summary answer Motility and vitality of fresh human sperm samples are significantly decreased under microgravity conditions obtained by parabolic flight. What is known already Microgravity effects on the male reproductive system have mainly been studied in the animal model with diverse results and discouraging extrapolation in humans. While an increased motility was reported in bulls, mice models showed a decrease. Although preliminary data from the Micro-11 experiment presented by NASA reported human sperm alterations after microgravity exposure, our first study failed to show any significant effect of microgravity on “frozen” samples, suggesting that human sperm could be safely shipped outside the earth if important aspects related with cryopreservation were solved. Study design, size, duration Prospective study carried out in collaboration between the ART centre, a Technical University, and an Aviation Club specializing in parabolic flights.Two parabolic flights were conducted between 2020-2021, each consisting of 20 parabolic maneuvers, which means 160 seconds of microgravity exposure per sample. Fifteen sperm samples obtained from healthy men were included in the study in order to analyse the effects of microgravity and compare the results with those obtained in Earth gravity. Participants/materials, setting, methods Fresh sperm samples were checked pre-flight to evaluate vitality, concentration, motility and morphology. Samples were split into two to compare the effects of different gravity exposure: microgravity (flight) and Earth gravity (ground). After the flight, the same analysis were repeated, plus kinematics, DNA fragmentation by sperm chromatin dispersion, apoptosis by magnetic activated cell sorting, and oxidative stress by colorimetric test (Halosperm-Halotech). Computer Aided Semen Analysis (SCA-Scope) was used for cell counting. Main results and the role of chance On comparison of the mean values between fresh samples exposed to microgravity and those maintained on Earth gravity, statistical significant differences (p < 0,05) were found in the following parameters: vitality (69,7 ± 9,9 vs 72,4 ± 9,7 %), motile sperm concentration (23,7 ± 15,3 M/ml vs 31,5 ± 25,1 M/ml), grade “a” sperm concentration (8,7 ± 6,5 vs 11,7 ± 9,9 M/ml), percentage of spermatozoa with progressive motility (30 ± 12,9 vs 36 ± 14,3 %), curvilinear motility-VCL (45,7 ± 12,8 vs 47,7 ± 13,3 μm/s). Under the study conditions, non-statistically significant differences were observed in the other kinematic parameters: Lineal Velocity (VSL), Average Path Velocity (VAP), Straightness (STR), Amplitude of Lateral Head displacement (ALH), Linearity (LIN), Wobble (WOB), Beat-Cross Frequency (BCF), total sperm concentration (81,7 ± 112,1 vs 79,7 ± 89,8 M/ml), morphology (11,3 ± 6,3 vs 10,6 ± 5,3%), DNA fragmentation (14,6 ± 9,6 vs 15,7 ± 9,4), apoptosis (2,8 ± 2,8 vs 3,8 ± 4,4) and oxidative stress, since all samples maintained the same stress level in both splits. Limitations, reasons for caution Parabolic flight is an accepted ground-based method for obtaining microgravity conditions, but provides a short period of elapsed exposure to microgravity. Therefore, the results obtained need to be confirmed by using other platforms that provide a much longer time of exposure. More cases must be analysed to confirm the results. Wider implications of the findings Short exposure to microgravity significantly decreases sperm motility and vitality. Such an effect is likely to be stronger with longer exposure. These findings should be taken into account since this may eventually affect sperm fertilizing capacity and therefore natural conception or ART with fresh/frozen sperm, outside of the Earth. Trial registration number NCT03760783

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