ETR-1, a homologue of a protein linked to myotonic dystrophy, is essential for muscle development in Caenorhabditis elegans.
Post-transcriptional gene processing by RNA-binding proteins (RBPs) has crucial roles during development [1] [2]. Here, we report the identification of ETR-1 (ELAV-type RNA-binding protein), a muscle-specific RBP in the nematode Caenorhabditis elegans. ETR-1 is related to the family of RBPs defined by the protein ELAV, which is essential for neurogenesis in the fruit fly Drosophila; members of the family possess two consecutive RNA recognition motifs (RRMs) separated from a third, carboxy-terminal RRM by a tether region of variable length [3] [4] [5] [6]. Its closest homologue, CUG-binding protein (CUG-bp), is a human RBP that has been implicated in the disease myotonic dystrophy and binds CUG repeats in the 3' untranslated region (UTR) of the mRNA for myotonic dystrophy protein kinase (DMPK) [7] [8]. Inactivation of etr-1 by RNA-mediated interference resulted in embryonic lethality. Embryos failed to elongate and became paralysed, a phenotype characteristic of C. elegans Pat mutants, which are defective in muscle formation and function [9]. The data indicate that etr-1 is essential for muscle development in C. elegans, perhaps by playing a role in post-transcriptional processing of some muscle component, and thus suggesting a possible conservation of gene function with human CUG-bp.
Milne
C A
CA
Medical Research Council, Laboratory of Molecular Biology, Hills Road, Cambridge, CB2 2QH, UK.
Hodgkin
J
J
D000818:Animals; D000067879:CELF1 Protein; D017173:Caenorhabditis elegans / Q000254:growth & development / Q000502:physiology*; D051959:ELAV Proteins; D006801:Humans; D024510:Muscle Development; D009124:Muscle Proteins / Q000302:isolation & purification / Q000502:physiology*; D009132:Muscles / Q000502:physiology; D009223:Myotonic Dystrophy / Q000378:metabolism*; D016601:RNA-Binding Proteins / Q000737:chemistry / Q000302:isolation & purification / Q000378:metabolism / Q000502:physiology*; D012261:Ribonucleoproteins / Q000737:chemistry / Q000378:metabolism; D029867:Xenopus Proteins
D000067879:CELF1 Protein; C102843:CELF1 protein, human; C094526:CELF3 protein, Xenopus; D051959:ELAV Proteins; D009124:Muscle Proteins; D016601:RNA-Binding Proteins; D012261:Ribonucleoproteins; D029867:Xenopus Proteins