Myelin deficiency in female rats due to a mutation in the PLP gene
| Autor: | K. D. Barron, Richard E. Dearborn, Arnulf H. Koeppen, Mogens Rønne, Ann M. Willey, Charles K. Csiza, Craig G. Hurwitz |
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| Rok vydání: | 1992 |
| Předmět: |
Male
Monosomy Proteolipid protein 1 X Chromosome Proteolipids Immunocytochemistry Myelin Compact myelin medicine Animals X chromosome Myelin Sheath Sexual differentiation biology Brain medicine.disease Molecular biology Immunohistochemistry Chromatin Myelin basic protein Rats Microscopy Electron medicine.anatomical_structure Phenotype nervous system Neurology Karyotyping Immunology Mutation biology.protein Electrophoresis Polyacrylamide Gel Female Neurology (clinical) Demyelinating Diseases |
| Zdroj: | Journal of the neurological sciences. 107(1) |
| ISSN: | 0022-510X |
| Popis: | Myelin deficiency ( md ) in female rats due to a mutation in the X-linked proteolipid protein (PLP) gene is caused by X-chromosome monosomy. Cytogenetic analysis revealed a single X karyotype [41,X(md/0)]. An immunocytochemical, electron microscopic, and biochemical study was performed on male and female md rats. The central nervous system (CNS) of the female md rat [41,X(md/0)] revealed the same total lack of PLP as the CNS of the affected male littermate [42,XY(md/Y)]. Immunocytochemistry for myelin basic protein (MBP), myelin-associated glycoprotein (MAG), and 2′,3′-cyclic nucleotide-3′-phosphodiesterase (CNP) revealed "islands" of myelin sheath-like reaction product in both. Electron microscopy showed great paucity of compact myelin sheaths in 41,X(md/0) and 42,XY(md/Y). Reduced levels of MPB, MAG, and CNP were confirmed for both sexes but MAG and CNP were substantially higher in 41,X(md/0). Sexual differentiation of the brain may account for the observed differences since normal female reproductive organs are present in the md female rat. |
| Databáze: | OpenAIRE |
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