The role of CELF proteins in neurological disorders.
CELF (CUG-BP and ETR-3-like factors) proteins are structurally related RNA-binding proteins involved in various aspects of RNA processing including splicing and mRNA stability. The first member of the family, CELF1/CUG-BP1, was identified through its role in myotonic dystrophy, type 1. Several recent studies have uncovered the recurrent implication, to various extents, of CELF proteins or of the functionally related muscleblind-like 1 protein in a number of neurological conditions. This is particularly clear for inherited neurodegenerative disorders caused by expansions of translated or untranslated triplet repeats in the causative gene. Here we review the role played by CELF proteins, at least as modifiers of the pathological phenotype, in a number of neurological diseases. The involvement of CELF proteins suggest that individual pathogenic pathways in a number of neurological conditions overlap at the level of RNA processing.
Gallo
Jean-Marc
JM
MRC Centre for Neurodegeneration Research, King's College London, Institute of Psychiatry, Department of Clinical Neuroscience, De Crespigny Park, London UK. jean-marc.gallo@kcl.ac.uk
Spickett
Carl
C
D000818:Animals; D020271:Heredodegenerative Disorders, Nervous System / Q000235:genetics; D006801:Humans; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D009422:Nervous System Diseases / Q000235:genetics*; D012323:RNA Processing, Post-Transcriptional; D016601:RNA-Binding Proteins / Q000378:metabolism*
D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins