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Artykuły w czasopismach na temat "Maladie de Farber"
Dalmat, Yann-Mickael. "Maladie de Farber, maladie pédiatrique". Option/Bio 29, nr 597-598 (maj 2019): 7. http://dx.doi.org/10.1016/s0992-5945(19)30098-4.
Pełny tekst źródłaOkusanya, P. O., O. O. Akinlade i M. I. Sangosina. "Survey on prevalent diseases in commercial poultry farms in Yewa South Local Government, Ogun State". Nigerian Journal of Animal Production 49, nr 1 (25.02.2022): 115–23. http://dx.doi.org/10.51791/njap.v49i1.3409.
Pełny tekst źródłaLevy, Brian J. "Malades et maladies dans les fabliaux". Reinardus / Yearbook of the International Reynard Society 12 (15.09.1999): 81–93. http://dx.doi.org/10.1075/rein.12.07lev.
Pełny tekst źródłaSerge Egide Paulin, Mensah, Sessou Philippe, Nata Christie, Adjahoutonon Koomlan Yélindo Kadjinou Brice, Lahamy Olivier i Farougou Souaïbou. "Mode d’utilisation des antibiotiques dans les élevages de bovin laitier sédentaires au nord-ouest du Bénin". Journal of Animal & Plant Sciences 42.2 (29.11.2019): 7198–206. http://dx.doi.org/10.35759/janmplsci.v42-2.2.
Pełny tekst źródłaM Joachim, Umba, Kumpel M. Placide, Maleke M. Jean-Pierre i Metena M. Marlène. "Evaluation environnementale et sanitaire des aires d’abattage à l’heure de Covid-19 situées dans la commune de Matete ville-province de Kinshasa (RD Congo)". Journal of Animal & Plant Sciences 46, nr 2 (30.11.2020): 8256–70. http://dx.doi.org/10.35759/janmplsci.v46-2.8.
Pełny tekst źródłaRozprawy doktorskie na temat "Maladie de Farber"
Denard, Jérôme. "Développement d'une approche de thérapie génique pour l'amyotrophie spinale avec épilepsie myoclonique progressive". Electronic Thesis or Diss., université Paris-Saclay, 2023. https://www.biblio.univ-evry.fr/theses/2023/interne/2023UPASL144.pdf.
Pełny tekst źródłaSpinal muscular atrophies are a heterogeneous group of disorders characterized by spinal cord α-motor neuron degeneration, leading muscle weakness and atrophy. The most common form is associated to mutations in the SMN1 gene, for which a gene therapy product obtained marketing approval. Another form of spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME), is due to mutations in the ASAH1 gene encoding a lysosomal enzyme,the acid ceramidase (ACDase). The life expectancy of patients with SMA-PME does not exceed adolescence. Some mutations in this same gene can cause Farber disease (FD), with the most severe forms causing death of patients before the age of 2 years due to severe multisystemic damage. SMA-PME and FD are ultra-rare lysosomal storage diseases. ACDase catalyzes the bioactive lipid ceramide into sphingosine and fatty acid. Due to the deficiency of enzyme activity, ceramides accumulate inside lysosomes causing serious dysfunctions in several organs. A therapeutic approach based on hematopoietic stem cell transplantation was previously tested in Farber patients but did not prevent neurological deterioration over time. The generation of mouse models of ACDase deficiency, in particular the Asah1P361R/P361R model that recapitulates the signs of Farber disease, has allowed a better understanding of the pathophysiology of acid ceramidase deficiency in tissues and the evaluation of different therapeutic approaches. Enzyme replacement therapy and gene therapy using a lentiviral vector extended the survival of mutant animals by several weeks, but did not correct the neurological signs of the disease. Since there is no curative treatment for patients, the main goal of my thesis project was to develop an efficacious gene therapy approach using an AAV vector. Intravenous administration of an AAV9-ASAH1 vector in Asah1P361R/P361R mice at pre- and symptomatic stages of the disease was able to prolong the lifespan and correct the phenotype. This approach provided proof of concept that generalized restoration of ASAH1 gene expression achieves a therapeutic effect in a severe mouse model of acid ceramidase deficiency. These results prompted us to perform a dose escalation study in Asah1P361R/P361R mice to determine the minimum effective dose of this vector. Three doses of AAV9-ASAH1 were administered intravenously in mutant mice at a late stage of the disease and the effect was analyzed at the clinical, molecular and histological level for a 6-month period. We identified a vector dose able to correct the phenotype and to avoid macrophage infiltrates in tissues, including the central nervous system. Intracerebroventricular administration (ICV) of the AAV9-ASAH1 vector in neonatal mutant mice was also evaluated to investigate the efficacy of this direct route of administration in correcting the neurological signs of the disease. The results show that systemic injection of the AAV9-ASAH1 vector was able to correct the whole-body phenotype of Asah1P361R/P361R mutant mice, whereas preliminary results indicate that ICV administration of the vector is efficacious in preventing mainly the neurological signs of the disease. This thesis work paves the way for clinical translation of this gene therapy in patients with Farber and SMA-PME diseases
Soname, S. O. O. "Effects of malaria on farmers' technical efficiency in Africa". Thesis, University of Reading, 2016. http://centaur.reading.ac.uk/66316/.
Pełny tekst źródłaROUX, CAIRE LAURENCE. "Diagnostic et traitement des maladies de l'articulation temporo-mandibulaire d'apres farrar et mc carthy". Aix-Marseille 2, 1988. http://www.theses.fr/1988AIX20264.
Pełny tekst źródłaMunthali, Grace Timanyechi. "Assessing farmers’ willingness-to-way for improved common bean seed varieties in Malawi : a case study of Kasungu and Dedza districts". Diss., University of Pretoria, 2013. http://hdl.handle.net/2263/40344.
Pełny tekst źródłaDissertation (MSc Agric)--University of Pretoria, 2013.
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Agricultural Economics, Extension and Rural Development
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Musonzo, Charity Priscilla. "Malawi Farm Input Subsidy Programme - impact on income of smallholder farmers". Master's thesis, University of Cape Town, 2015. http://hdl.handle.net/11427/29044.
Pełny tekst źródłaCanz, Stefan. "Linking small scale farmers to markets : a multi-level analysis with special references to Malawi, Kenya and South Africa /". Weikersheim Margraf, 2005. http://deposit.ddb.de/cgi-bin/dokserv?id=2686372&prov=M&dok_var=1&dok_ext=htm.
Pełny tekst źródłaChiwona-Karltun, Linley. "A reason to be bitter : cassava classification from the farmers' perspective /". Stockholm : [Karolinska institutets bibl.], 2001. http://diss.kib.ki.se/2001/91-7349-078-4/.
Pełny tekst źródłaMwangwela, Dennis Lusekelo. "Towards appropriate institutional linkage structures for effective participatory and coordinated agricultural extension in Malawi". Diss., Pretoria : [s.n.], 2006. http://upetd.up.ac.za/thesis/available/etd-07032007-094422.
Pełny tekst źródłaMazzone, Angelica <1992>. "New advances in molecular epidemiology of emerging enteric parasites in farmed gilthead sea bream (Sparus aurata) and insights into zoonotic parasites of new relevance in Italian freshwater fish". Doctoral thesis, Alma Mater Studiorum - Università di Bologna, 2021. http://amsdottorato.unibo.it/9665/1/angelica_mazzone_tesi.pdf.
Pełny tekst źródłaVuong, Thao Thi Phuong. "Farmers' perceptions of the "Unleashing the Power of Cassava in Africa in Response to the Food Crisis" (UPoCA) project : Experiences from Malawi". Thesis, Uppsala universitet, Institutionen för geovetenskaper, 2012. http://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-171455.
Pełny tekst źródłaKsiążki na temat "Maladie de Farber"
Bekunda, Mateete, Irmgard Hoeschle-Zeledon i Jonathan Odhong, red. Sustainable agricultural intensification: a handbook for practitioners in East and Southern Africa. Wallingford: CABI, 2022. http://dx.doi.org/10.1079/9781800621602.0000.
Pełny tekst źródłaHealth for the Farmer. Farming Press Limited, 1991.
Znajdź pełny tekst źródłaMvula, Peter, i Wapulumuka Mulwafu. Intensification, Crop Diversification, and Gender Relations in Malawi. Oxford University Press, 2018. http://dx.doi.org/10.1093/oso/9780198799283.003.0007.
Pełny tekst źródłaPollack, Howard. Banjo Eyes. Oxford University Press, 2017. http://dx.doi.org/10.1093/oso/9780190458294.003.0009.
Pełny tekst źródłaCzęści książek na temat "Maladie de Farber"
Wahl, Carl. "Lessons learnt from concern worldwide's conservation agriculture interventions in Malawi and Zambia, 2010-2018." W Conservation agriculture in Africa: climate smart agricultural development, 361–70. Wallingford: CABI, 2022. http://dx.doi.org/10.1079/9781789245745.0022.
Pełny tekst źródłaWellard, Kate, Daimon Kambewa i Sieglinde Snapp. "Farmers on the frontline: adaptation and change in Malawi". W Climate Change and Threatened Communities, 41–56. Rugby, Warwickshire, United Kingdom: Practical Action Publishing, 2012. http://dx.doi.org/10.3362/9781780447254.004.
Pełny tekst źródłaMuhonda, Park. "Smallholder farmers' lived experiences of weather perturbation in Malawi". W The Routledge Handbook of Development and Environment, 145–60. London: Routledge, 2021. http://dx.doi.org/10.4324/9780429450310-15.
Pełny tekst źródłaChitika, Rollins. "9. Rice – smallholder farmers in Malawi can be profitably included". W Commercial and Inclusive Value Chains, 89–95. The Schumacher Centre, Bourton on Dunsmore, Rugby, Warwickshire CV23 9QZ, UK,: Practical Action Publishing Ltd, 2015. http://dx.doi.org/10.3362/9781780448671.009.
Pełny tekst źródłaTembo-Nhlema, Dorothy, Katharine Vincent i Rebecka Henriksson. "Creating Useful and Usable Weather and Climate Information: Insights from Participatory Scenario Planning in Malawi". W Climate Risk in Africa, 77–92. Cham: Springer International Publishing, 2021. http://dx.doi.org/10.1007/978-3-030-61160-6_5.
Pełny tekst źródłaZimba, Josephine M., i Emma T. Liwenga. "Effects of conservation agriculture on farmers' livelihoods in the face of climate change in Balaka district, Malawi." W Climate change impacts and sustainability: ecosystems of Tanzania, 44–58. Wallingford: CABI, 2020. http://dx.doi.org/10.1079/9781789242966.0044.
Pełny tekst źródłaMoyo, Mukani, Van-Den Truong, Josip Simunovic, Jean Pankuku, George Ooko Abong, Francis Kweku Amagloh, Richard Fuchs i in. "Orange-Fleshed Sweetpotato Puree: A Breakthrough Product for the Bakery Sector in Africa". W Root, Tuber and Banana Food System Innovations, 145–72. Cham: Springer International Publishing, 2022. http://dx.doi.org/10.1007/978-3-030-92022-7_5.
Pełny tekst źródłaKanyamuka, Joseph S., Charles B. L. Jumbe, Jacob Ricker-Gilbert, Abdi-Khalil Edriss i Wezi G. Mhango. "Determinants of ISFM Technology Adoption and Disadoption Among Smallholder Maize Farmers in Central Malawi". W Climate Impacts on Agricultural and Natural Resource Sustainability in Africa, 449–69. Cham: Springer International Publishing, 2020. http://dx.doi.org/10.1007/978-3-030-37537-9_26.
Pełny tekst źródłaKerr, Rachel Bezner, Rodgers Msachi, Laifolo Dakishoni, Lizzie Shumba, Zachariah Nkhonya, Peter Berti, Christine Bonatsos i in. "Growing Healthy Communities: Farmer Participatory Research to Improve Child Nutrition, Food Security, and Soils in Ekwendeni, Malawi". W Ecohealth Research in Practice, 37–46. New York, NY: Springer New York, 2011. http://dx.doi.org/10.1007/978-1-4614-0517-7_3.
Pełny tekst źródłaMsalya, G., Z. C. Nziku, T. Gondwe, G. C. Kifaro, L. O. Eik i T. Ådnøy. "The Need for Farmer Support and Record Keeping to Enhance Sustainable Dairy Goat Breeding in Tanzania and Malawi". W Climate Impacts on Agricultural and Natural Resource Sustainability in Africa, 287–99. Cham: Springer International Publishing, 2020. http://dx.doi.org/10.1007/978-3-030-37537-9_17.
Pełny tekst źródłaStreszczenia konferencji na temat "Maladie de Farber"
Cai, Tian, Hastings Chiwasa, Charles Steinfield i Susan Wyche. "Participatory video for nutrition training for farmers in Malawi". W the Seventh International Conference. New York, New York, USA: ACM Press, 2015. http://dx.doi.org/10.1145/2737856.2737878.
Pełny tekst źródłaMoyo, Yotam Mgonjetsi, Mohamed F. Jeebhay, Roslynn Baatjies, Sufia Dadabhai i Shahieda Adams. "O-213 Obstructive lung disease associated with occupational and environmental exposures among small-scale tobacco farmers in Malawi". W 29th International Symposium on Epidemiology in Occupational Health (EPICOH 2023), Mumbai, India, Hosted by the Indian Association of Occupational Health, Mumbai Branch & Tata Memorial Centre. BMJ Publishing Group Ltd, 2023. http://dx.doi.org/10.1136/oem-2023-epicoh.50.
Pełny tekst źródłaRaporty organizacyjne na temat "Maladie de Farber"
Khaila, Stanley, Frank Tchuwa, Steven Franzel i Brent Simpson. The Farmer-to-Farmer Extension Approach in Malawi: A Survey of Lead Farmers. World Agroforestry Centre, 2015. http://dx.doi.org/10.5716/wp14152.pdf.
Pełny tekst źródłaKhaila, S., F. Tchuwa, Steve Franzel i B. Simpson. The farmer-to-farmer extension approach in Malawi: a survey of lead farmers. World Agroforestry Centre (ICRAF), 2015. http://dx.doi.org/10.5716/wp14200.pdf.
Pełny tekst źródłaChimombo, Masautso, Mirriam Matita, Loveness Mgalamadzi, Blessings Chinsinga, Ephraim Wadonda Chirwa, Stevier Kaiyatsa i Jacob Mazalale. Interrogating the Effectiveness of Farmer Producer Organisations in Enhancing Smallholder Commercialisation – Frontline Experiences From Central Malawi. Institute of Development Studies (IDS), luty 2022. http://dx.doi.org/10.19088/apra.2022.004.
Pełny tekst źródłaResearch Institute (IFPRI), International Food Policy. The impact of a farmer business school program on incomes of smallholder farmers: Insights from central Malawi. Washington, DC: International Food Policy Research Institute, 2018. http://dx.doi.org/10.2499/1037800837.
Pełny tekst źródłaKundhlande, Godfrey, Steven Franzel, Brent Simpson i Emma Gausi. Farmer-to-farmer extension approach in Malawi: A survey of organizations using the approach. World Agroforestry Centre, 2014. http://dx.doi.org/10.5716/wp14384.pdf.
Pełny tekst źródłaMatita, Mirriam, Matita, Mirriam, Ephraim Wadonda Chirwa, Stevier Kaiyatsa, Jacob Mazalale, Masautso Chimombo, Loveness Msofi Mgalamadzi i Blessings Chinsinga. Determinants of Smallholder Farmers’ Livelihood Trajectories: Evidence from Rural Malawi. Institute of Development Studies (IDS), luty 2021. http://dx.doi.org/10.19088/apra.2021.003.
Pełny tekst źródłaMatita, Mirriam, Ephraim Wadonda Chirwa, David Zingwe i Jacob Mazalale. Use of Climate-Smart Agriculture Practices and Smallholder Farmer Market Participation in Central Malawi. Institute of Development Studies (IDS), luty 2022. http://dx.doi.org/10.19088/apra.2022.003.
Pełny tekst źródłaChinsinga, Blessings, Mirriam Matita, Masautso Chimombo, Loveness Msofi, Stevier Kaiyatsa i Jacob Mazalale. Agricultural Commercialisation and Rural Livelihoods in Malawi: A Historical and Contemporary Agrarian Inquiry. Institute of Development Studies (IDS), listopad 2021. http://dx.doi.org/10.19088/apra.2021.043.
Pełny tekst źródłaMsukwa, Chimwemwe, Jane Burt i John Colvin. Good Governance in Malawi: Impact evaluation of the ‘Strengthening Land Governance System for Smallholder Farmers in Malawi’ project. Oxfam GB, marzec 2021. http://dx.doi.org/10.21201/2021.7345.
Pełny tekst źródłaNindi, Tabitha. Understanding the factors that influence cereal-legume adoption amongst smallholder farmers in Malawi. Washington, DC: International Food Policy Research Institute, 2021. http://dx.doi.org/10.2499/p15738coll2.134316.
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