| RRC ID |
27738
|
| 著者 |
Tanaka M, Soma K, Izawa T, Yamate J, Franklin RJ, Kuramoto T, Serikawa T, Kuwamura M.
|
| タイトル |
Abnormal myelinogenesis in the central nervous system of the VF mutant rat with recoverable tremor.
|
| ジャーナル |
Brain Res
|
| Abstract |
The vacuole formation (VF) rat is an autosomal recessive myelin mutant characterized by generalized tremor and vacuole formation within the myelin of the central nervous system (CNS). The tremor is prominent between 4 and 8 weeks of age but thereafter gradually improves. The present study, aimed at producing a detailed description of how the principal lesions evolve, revealed that the VF rat has abnormal vacuoles in the periaxonal space in the spinal cord and that these vacuoles decreased concurrently with the loss of tremor. However, a degree of hypomyelination persisted throughout the CNS after the resolution of periaxonal vacuoles. In situ hybridization for proteolipid protein (PLP) demonstrated that the number of small, round PLP-positive oligodendrocytes increased in the spinal cord of VF rats between 10 and 20 weeks of age, suggesting a disruption of oligodendrocytes' maturation. Activated astrocytes and microglia were also found in the VF rats from 4 weeks of age. Our results indicate that the causative genetic defect of the VF rat is likely to be involved in the formation and maintenance of the CNS myelin.
|
| 巻・号 |
1488
|
| ページ |
104-12
|
| 公開日 |
2012-12-7
|
| DOI |
10.1016/j.brainres.2012.09.037
|
| PII |
S0006-8993(12)01578-8
|
| PMID |
23036276
|
| MeSH |
Animals
Astrocytes / pathology
Astrocytes / ultrastructure
Demyelinating Diseases / genetics*
Demyelinating Diseases / pathology*
Disease Models, Animal
Microglia / pathology
Microglia / ultrastructure
Microscopy, Electron
Myelin Sheath / pathology*
Myelin Sheath / ultrastructure
Nerve Fibers, Myelinated / pathology
Nerve Fibers, Myelinated / ultrastructure
Oligodendroglia / pathology
Oligodendroglia / ultrastructure
Rats
Rats, Inbred Strains
Rats, Mutant Strains
Tremor / genetics*
Tremor / pathology*
Vacuoles / pathology*
Vacuoles / ultrastructure
|
| IF |
2.733
|
| 引用数 |
5
|
|
WOS 分野
|
NEUROSCIENCES
|
| リソース情報 |
| ラット |
VF/Kyo(strainID=21) |
