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 recen...

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Bibliographic Details
Published in:RNA biology 2010-07, Vol.7 (4), p.474-479
Main Authors: Gallo, Jean-Marc, Spickett, Carl
Format: Article
Language:English
Subjects:
Animals
Binding
Biology
Bioscience
Calcium
Cancer
Cell
Cycle
Heredodegenerative Disorders, Nervous System - genetics
Humans
Landes
Nerve Tissue Proteins - metabolism
Nervous System Diseases - genetics
Organogenesis
Proteins
Review
RNA Processing, Post-Transcriptional
RNA-Binding Proteins - metabolism
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Summary: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.
ISSN:1547-6286
1555-8584
DOI:10.4161/rna.7.4.12345