| Inner ear defect similar to Alport's syndrome in the glomerulosclerosis mouse model Mpv17. | |
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MedLine Citation:
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PMID: 9044568 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The Mpv17 mouse strain is a recessive transgenic mouse mutant that develops glomerulosclerosis and nephrotic syndrome at a young age. The phenotype results from a loss of function of a gene coding for a hydrophobic peroxisomal protein of 176 amino acids of 20 kDa following its destruction by retroviral integration. To investigate a potential effect of the missing Mpv17 function on the inner ear light and electron microscopic investigations were performed on the inner ears of Mpv17 mice and controls. These revealed degeneration of the stria vascularis and spiral ligament, loss of cochlear neurons and degeneration of the organ of Corti. The alterations observed here were similar to those described for Alport's syndrome, an inherited disorder characterized by progressive nephritis and neurosensory deafness. These findings indicate that although the molecular cause is different, the Mpv17 mouse model may share pathological mechanisms involved in patients with Alport's syndrome. At present the Mpv17 mouse appears to be a suitable animal model for this disease and may help to further elucidate the relationship between the kidney and the inner ear. |
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Authors:
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A M Meyer zum Gottesberge; A Reuter; H Weiher |
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Publication Detail:
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Type: Comparative Study; Journal Article |
Journal Detail:
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Title: European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery Volume: 253 ISSN: 0937-4477 ISO Abbreviation: Eur Arch Otorhinolaryngol Publication Date: 1996 |
Date Detail:
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Created Date: 1997-03-04 Completed Date: 1997-03-04 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 9002937 Medline TA: Eur Arch Otorhinolaryngol Country: GERMANY |
Other Details:
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Languages: eng Pagination: 470-4 Citation Subset: IM |
Affiliation:
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Department of ORL, Heinrich Heine University, Düsseldorf, Germany. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Disease Models, Animal* Ear, Inner / abnormalities*, ultrastructure Female Glomerulosclerosis, Focal Segmental / genetics, pathology* Kidney / ultrastructure Male Mice Mice, Transgenic Microscopy, Electron Nephritis, Hereditary / genetics, pathology* Organ of Corti / ultrastructure Phenotype |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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