Inactivation of mediator complex protein 22 in podocytes results in intracellular vacuole formation, podocyte loss and premature death
| العنوان: | Inactivation of mediator complex protein 22 in podocytes results in intracellular vacuole formation, podocyte loss and premature death |
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| المؤلفون: | Katja Möller-Hackbarth, Jaakko Patrakka, Timo Jahnukainen, David Unnersjö-Jess, Lwaki Ebarasi, Sonia Zambrano, Jing Guo, Patricia Q. Rodriguez, Hans Blom |
| المساهمون: | HUS Children and Adolescents, Children's Hospital, Helsinki University Hospital Area, University of Helsinki |
| المصدر: | Scientific Reports, Vol 10, Iss 1, Pp 1-12 (2020) Scientific Reports |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | Adult, EXPRESSION, Molecular biology, Kidney Glomerulus, lcsh:Medicine, Vacuole, Biology, Article, Podocyte, 03 medical and health sciences, Mice, 0302 clinical medicine, Mediator, KIDNEY, GLOMERULI, medicine, Animals, Humans, lcsh:Science, 030304 developmental biology, Mice, Knockout, 0303 health sciences, Kidney, Multidisciplinary, Mediator Complex, IDENTIFICATION, Mortality, Premature, Podocytes, lcsh:R, COMPONENTS, Nephrons, SUBUNITS, 3. Good health, Cell biology, Ultrafiltration (renal), medicine.anatomical_structure, Mechanisms of disease, Nephrology, 030220 oncology & carcinogenesis, Renal physiology, 3121 General medicine, internal medicine and other clinical medicine, Knockout mouse, Vacuoles, Kidney Diseases, lcsh:Q, Intracellular |
| الوصف: | Podocytes are critical for the maintenance of kidney ultrafiltration barrier and play a key role in the progression of glomerular diseases. Although mediator complex proteins have been shown to be important for many physiological and pathological processes, their role in kidney tissue has not been studied. In this study, we identified a mediator complex protein 22 (Med22) as a renal podocyte cell-enriched molecule. Podocyte-specific Med22 knockout mouse showed that Med22 was not needed for normal podocyte maturation. However, it was critical for the maintenance of podocyte health as the mice developed progressive glomerular disease and died due to renal failure. Detailed morphological analyses showed that Med22-deficiency in podocytes resulted in intracellular vacuole formation followed by podocyte loss. Moreover, Med22-deficiency in younger mice promoted the progression of glomerular disease, suggesting Med22-mediated processes may have a role in the development of glomerulopathies. This study shows for the first time that mediator complex has a critical role in kidney physiology. |
| اللغة: | English |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ba6891d51735e4fb4c10d202dfcb635b http://hdl.handle.net/10138/325875 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....ba6891d51735e4fb4c10d202dfcb635b |
| قاعدة البيانات: | OpenAIRE |
| الوصف غير متاح. |