Hemicentin, a conserved extracellular member of the immunoglobulin superfamily, organizes epithelial and other cell attachments into oriented line-shaped junctions.
him-4 mutations cause a novel syndrome of tissue fragility, defective cell migration and chromosome instability in Caenorhabditis elegans. Null mutants have abnormal escape reflex, mispositioning of the vas deferens and uterus, and mitotic chromosome loss and multinucleate cells in the germline. The him-4 gene product, hemicentin, is a conserved extracellular matrix protein with 48 tandem immunoglobulin repeats flanked by novel terminal domains. Secreted from skeletal muscle and gonadal leader cells, hemicentin assembles into fine tracks at specific sites, where it contracts broad regions of cell contact into oriented linear junctions. Some tracks organize hemidesmosomes in the overlying epidermis. Hemicentin tracks facilitate mechanosensory neuron anchorage to the epidermis, gliding of the developing gonad along epithelial basement membranes and germline cellularization.
Vogel
B E
BE
Department of Biology, Johns Hopkins University, Baltimore, MD 21218, USA. vogel@umbi.umd.edu
Hedgecock
E M
EM
D000595:Amino Acid Sequence; D000782:Aneuploidy; D000818:Animals; D030801:Animals, Genetically Modified; D017173:Caenorhabditis elegans / Q000235:genetics* / Q000502:physiology; D029742:Caenorhabditis elegans Proteins; D002448:Cell Adhesion; D012734:Disorders of Sex Development; D004847:Epithelial Cells / Q000502:physiology; D016326:Extracellular Matrix Proteins / Q000235:genetics / Q000502:physiology; D005260:Female; D017930:Genes, Reporter; D049452:Green Fluorescent Proteins; D015801:Helminth Proteins / Q000235:genetics / Q000502:physiology*; D007136:Immunoglobulins / Q000737:chemistry / Q000235:genetics*; D008164:Luminescent Proteins / Q000235:genetics; D008297:Male; D008565:Membrane Proteins / Q000235:genetics / Q000502:physiology*; D008969:Molecular Sequence Data; D021382:Protein Sorting Signals / Q000235:genetics; D016415:Sequence Alignment; D017386:Sequence Homology, Amino Acid; D014599:Uterus / Q000502:physiology; D014649:Vas Deferens / Q000502:physiology; D014960:X Chromosome
D029742:Caenorhabditis elegans Proteins; D016326:Extracellular Matrix Proteins; D015801:Helminth Proteins; D007136:Immunoglobulins; D008164:Luminescent Proteins; D008565:Membrane Proteins; D021382:Protein Sorting Signals; C424064:him-4 protein, C elegans; D049452:Green Fluorescent Proteins