The QKI-6 and QKI-7 RNA binding proteins block proliferation and promote Schwann cell myelination.

ABSTRACT:

Background

The quaking viable (qk(v)) mice have uncompacted myelin in their central and peripheral nervous system (CNS, PNS). The qk gene encodes 3 major alternatively spliced isoforms that contain unique sequence at their C-terminus dictating their cellular localization. QKI-5 is a nuclear isoform, whereas QKI-6 and QKI-7 are cytoplasmic isoforms. The qk(v) mice harbor an enhancer/promoter deletion that prevents the expression of isoforms QKI-6 and QKI-7 in myelinating cells resulting in a dysmyelination phenotype. It was shown that QKI regulates the differentiation of oligodendrocytes, the myelinating cells of the CNS, however, little is known about the role of the QKI proteins, or RNA binding proteins in PNS myelination.

Methodology/principal findings

To define the role o

SUBMITTER: Larocque D 

PROVIDER: S-EPMC2690695 | biostudies-literature | 2009 Jun

REPOSITORIES: biostudies-literature