First Author | Hardy RJ | Year | 1996 |
Journal | J Neurosci | Volume | 16 |
Issue | 24 | Pages | 7941-9 |
PubMed ID | 8987822 | Mgi Jnum | J:37139 |
Mgi Id | MGI:84567 | Doi | 10.1523/JNEUROSCI.16-24-07941.1996 |
Citation | Hardy RJ, et al. (1996) Neural cell type-specific expression of QKI proteins is altered in quakingviable mutant mice. J Neurosci 16(24):7941-9 |
abstractText | qkI, a newly cloned gene lying immediately proximal to the deletion in the quakingviable mutation, is transcribed into three messages of 5, 6, and 7 kb. Antibodies raised to the unique carboxy peptides of the resulting QKI proteins reveal that, in the nervous system, all three QKI proteins are expressed strongly in myelin-forming cells and also in astrocytes. Interestingly, individual isoforms show distinct intracellular distributions: QKI-6 and QKI-7 are localized to perikaryal cytoplasm, whereas QKI-5 invariably is restricted to the nucleus, consistent with the predicted role of QKI as an RNA-binding protein. In quakingviable mutants, which display severe dysmyelination, QKI-6 and QKI-7 are absent exclusively from myelin-forming cells. By contrast, QKI-5 is absent only in oligodendrocytes of severely affected tracts. These observations implicate QKI proteins as regulators of myelination and reveal key insights into the mechanisms of dysmyelination in the quakingviable mutant. |