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Relevant bibliographies by topics / Wobbler mice

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Academic literature on the topic 'Wobbler mice'

Author: Grafiati

Published: 9 March 2023

Last updated: 10 March 2023

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Journal articles on the topic "Wobbler mice"

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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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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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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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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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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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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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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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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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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Dissertations / Theses on the topic "Wobbler mice"

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Bose, Prodip Kumar. "Wobbler mouse: early detection of motoneuron disease, therapeutic evaluation of nutrition, neuropeptides & theirantagonists, and the effects on neuronal sprouting in cervical spinalcord." Thesis, The University of Hong Kong (Pokfulam, Hong Kong), 1997. http://hub.hku.hk/bib/B31236522.

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Bose, Prodip Kumar. "Wobbler mouse : early detection of motoneuron disease, therapeutic evaluation of nutrition, neuropeptides & their antagonists, and the effects on neuronal sprouting in cervical spinal cord /." Hong Kong : University of Hong Kong, 1997. http://sunzi.lib.hku.hk/hkuto/record.jsp?B19118168.

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Ripolone, M. "mUBPY, endocytic vesicle traffic and microtubule mediated transport in acrosome biogenesis. Comparative study between wild-type and Wobbler (L967Q Vps54) mice." Doctoral thesis, Università degli Studi di Milano, 2009. http://hdl.handle.net/2434/140980.

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Acrosome biogenesis is a multistep process, essential for fertilization, involving poorly understood events, like vesicular trafficking and microtubular transport. We found that the deubiquitinating enzyme UBPy, in mouse, marks step-by-step the development of the acrosomal vesicle. Recently by studies on transfected cells, UBPy is emerging to play a key and no redundant role in endosomal sorting, resulting to be fundamental for the cell life. In this work we studied in male germ cells the endosomal trafficking machinery, in particular we detected the early endosome compartment and the ESCRT-0 complex, interestingly mUBPy colocalizes and interacts with Hrs and Hbp (Hrs-binding protein) at the early endosomes. We investigated also Vps54, a component of the Golgi associated retrograde protein complex which is involved in vesicles transport from early endosomes to trans Golgi network. Altogether our findings demonstred for the first time the presence of a mutiprotein complex in spermatogenic cells. Interestingly, proteins belonging to vesicular compartment participate and cooperate with UBPy to the formation of the acrosome, and here remain in the mature sperm, strongly suggesting that the endosome system, through an UBPy-microtubular manchette-mediated transport, could contribute to the biogenesis of the acrosome. The missense mutation L967Q in Vps54 marks the wobbler mouse phenotype. Wobbler mouse is an animal model for motor neuron neurodegenerative disorders with associated male sterility. We provided the first characterization of wobbler reproductive apparatus, detecting the absence of the acrosome, an abnormal shape of sperm s head and a defective vesicular transport.

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Yung, Kin-lam Ken. "Quantification of neuropeptides in the central nervous system of the wobbler mouse during the progression of the motor neuron disease : a study by radioimmunoassay and immunocytochemistry /." [Hong Kong : University of Hong Kong], 1992. http://sunzi.lib.hku.hk/hkuto/record.jsp?B1320502X.

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翁建霖 and Kin-lam Ken Yung. "Quantification of neuropeptides in the central nervous system of the wobbler mouse during the progression of the motor neuron disease: a study by radioimmunoassay andimmunocytochemistry." Thesis, The University of Hong Kong (Pokfulam, Hong Kong), 1992. http://hub.hku.hk/bib/B31210673.

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CANZI, LAURA. "Human stem cells for the treatment of motorneuron diseases: regenerative potential, translatability and development of new biotechnologies. Cellule staminali umane per la cura delle malattie degenerative del motoneurone." Doctoral thesis, Università degli Studi di Milano-Bicocca, 2010. http://hdl.handle.net/10281/19217.

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Stem cell therapy is considered one of the most promising approaches against different neurodegenerative disorders, including Amyotorophic Lateral Sclerosis (ALS). The evidence that the systemic injection of human cord blood mononuclear cells (HuCB-MNC) was able to reduce the clinical outcomes and increase the lifespan in a murine model of fALS1, the SOD1G93A mouse, even if localized far from affected motor neurons, opens the way for new possible candidates and alternative ways of administration. Here the effect of human skeletal muscle-derived stem cell (SkmSCs) was investigated by single administration in lateral ventricles in the most characterized model of spontaneous motor neuron degeneration, the Wobbler (Wr) mouse. Before evaluating clinical progression, we found that SkmSCs (previously labeled with the super paramagnetic contrast agent Endorem™ and/or with the fluorescent nuclear dye Hoeschst 33258): 1) spread along the whole ventricular system as far as the ependymal canal at the spinal cord level; 2) remained for a longer time in the Wr than in the healthy mice, and; 3) did not significantly migrate to the parenchyma. Similar to the SOD1G93A mice treated with HuCB-MNCs, the transplantation of SkmSCs: 1) significantly improved the disease progression of ALS-related Wr motorneuropathology; 2) this effect was not associated with a migration of SkmSCs close to the degenerating motor neurons. Very interestingly, we also found that cell grafting in the Wr brain ventricles significantly increased the gene expression of anti-inflammatory cytokines or chemokines activated in the inflammatory response. These results further confirm the consistency of the hypothesis of the bystander effect of stem cells in motor neurodegenerative disorders by a mechanism of action aimed at reducing the neuroinflammatory response.

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Conference papers on the topic "Wobbler mice"

1

Wickstrom, Eric, Chang-Po Chen, Matthew E. Wampole, Kaijun Zhang, Bishnuhari Paudyal, Antican Wang, Sushil Tripathi, et al. "Abstract B55: Hypoxanthine wobble base multimutant KRAS2 mRNA PET imaging agent in G12D mice." In Abstracts: AACR Special Conference on RAS Oncogenes: From Biology to Therapy; February 24-27, 2014; Lake Buena Vista, FL. American Association for Cancer Research, 2014. http://dx.doi.org/10.1158/1557-3125.rasonc14-b55.

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