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Genetics ; 203(4): 1789-806, 2016 08.
Artículo en Inglés | MEDLINE | ID: mdl-27334269

RESUMEN

Determination of luminal diameter is critical to the function of small single-celled tubes. A series of EXC proteins, including EXC-1, prevent swelling of the tubular excretory canals in Caenorhabditis elegans In this study, cloning of exc-1 reveals it to encode a homolog of mammalian IRG proteins, which play roles in immune response and autophagy and are associated with Crohn's disease. Mutants in exc-1 accumulate early endosomes, lack recycling endosomes, and exhibit abnormal apical cytoskeletal structure in regions of enlarged tubules. EXC-1 interacts genetically with two other EXC proteins that also affect endosomal trafficking. In yeast two-hybrid assays, wild-type and putative constitutively active EXC-1 binds to the LIM-domain protein EXC-9, whose homolog, cysteine-rich intestinal protein, is enriched in mammalian intestine. These results suggest a model for IRG function in forming and maintaining apical tubule structure via regulation of endosomal recycling.


Asunto(s)
Proteínas de Caenorhabditis elegans/genética , Endosomas/genética , Metaloproteínas/genética , Animales , Autofagia/genética , Caenorhabditis elegans/genética , Caenorhabditis elegans/crecimiento & desarrollo , Proteínas de Caenorhabditis elegans/metabolismo , Enfermedad de Crohn/genética , Enfermedad de Crohn/patología , Citoesqueleto/genética , Citoesqueleto/metabolismo , Endosomas/metabolismo , Humanos , Túbulos Renales/crecimiento & desarrollo , Túbulos Renales/metabolismo , Metaloproteínas/metabolismo , Transporte de Proteínas/genética , Técnicas del Sistema de Dos Híbridos
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