Molecular biology of myelin basic protein: Gene rearrangement and expression of anti-sense RNA in myelin-deficient mutants

Mikoshiba Katsuhiko, Aruga Jun, Okano Hideyuki

研究成果: Article査読

7 被引用数 (Scopus)

抄録

1. Myelin is an important structure for facilitating the conduction of impulses along the axons both in the central nervous system (CNS) and peripheral nervous system (PNS). 2. Myelin basic protein (MBP) is a major protein in CNS myelin. 3. MBP is expressed specifically in the nervous system. 4. The MBP gene has been cloned and characterized. 5. Two mutant mice, Shiverer (shi) and myelin-deficient (mld, shimid), are autosomal recessive mutants that show severe symptoms such as intentional tremor. They have been found to have a mutation in the MBP gene that results in poor myelination in the central nervous system. 6. It was found that rearrangement within the MBP gene results in low expression of the gene. 7. In Shiverer, the MBP gene is partially deleted (from exons 3 to 7), and in mld, the gene is duplicated tandemly and a large portion of the duplication is inverted upstream of the intact copy. 8. In mld, anti-sense RNA complementary to exons 3-7, which correspond to the inverted segment, was detected by RNase protection studies, and presumed to be responsible for the reduced expressions of MBP. 9.The mechanism of gene rearrangement in MBP was also characterized. 10. This article reviews the recent progress in the study of the MBP gene, especially the rearrangement of the gene and its expression in mutant mice.

本文言語English
ページ(範囲)51-61
ページ数11
ジャーナルComparative Biochemistry and Physiology. Part C, Comparative
98
1
DOI
出版ステータスPublished - 1991
外部発表はい

ASJC Scopus subject areas

  • 免疫学
  • 薬理学

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