UNC-39, the C. elegans homolog of the human myotonic dystrophy-associated homeodomain protein Six5, regulates cell motility and differentiation.
Mutations in the unc-39 gene of C. elegans lead to migration and differentiation defects in a subset of mesodermal and ectodermal cells, including muscles and neurons. Defects include mesodermal specification and differentiation as well a neuronal migration and axon pathfinding defects. Molecular analysis revealed that unc-39 corresponds to the previously named gene ceh-35 and that the UNC-39 protein belongs to the Six4/5 family of homeodomain transcription factors and is similar to human Six5, a protein implicated in the pathogenesis of type I myotonic dystrophy (DM1). We show that human Six5 and UNC-39 are functional homologs, suggesting that further characterization of the C. elegans unc-39 gene might provide insight into the etiology of DM1.
Department of Embryology, Carnegie Institution of Washington, Baltimore, MD 21210, USA. jly@alum.mit.edu
Judith L
JL
Yanowitz
M Afaq
MA
Shakir
Edward
E
Hedgecock
Harald
H
Hutter
Andrew Z
AZ
Fire
Erik A
EA
Lundquist
D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D017173:Caenorhabditis elegans / Q000166:cytology* / Q000196:embryology; D029742:Caenorhabditis elegans Proteins / Q000235:genetics* / Q000378:metabolism*; D002454:Cell Differentiation / Q000502:physiology*; D002465:Cell Movement / Q000502:physiology; D002610:Cheek / Q000254:growth & development; D004475:Ectoderm / Q000502:physiology; D004625:Embryo, Nonmammalian; D004627:Embryonic Induction; D005260:Female; D018507:Gene Expression Regulation, Developmental; D006257:Head / Q000196:embryology; D018398:Homeodomain Proteins / Q000235:genetics* / Q000378:metabolism*; D006801:Humans; D008648:Mesoderm / Q000473:pathology; D008969:Molecular Sequence Data; D009132:Muscles / Q000166:cytology / Q000196:embryology; D009154:Mutation; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D010614:Pharynx / Q000254:growth & development; D017386:Sequence Homology, Amino Acid; D014844:Vulva / Q000166:cytology / Q000196:embryology
D029742:Caenorhabditis elegans Proteins; D018398:Homeodomain Proteins; C106687:SIX5 protein, human; C491439:Unc-39 protein, C elegans