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Genetics ; 203(4): 1789-806, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27334269

RESUMO

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.


Assuntos
Proteínas de Caenorhabditis elegans/genética , Endossomos/genética , Metaloproteínas/genética , Animais , Autofagia/genética , Caenorhabditis elegans/genética , Caenorhabditis elegans/crescimento & desenvolvimento , Proteínas de Caenorhabditis elegans/metabolismo , Doença de Crohn/genética , Doença de Crohn/patologia , Citoesqueleto/genética , Citoesqueleto/metabolismo , Endossomos/metabolismo , Humanos , Túbulos Renais/crescimento & desenvolvimento , Túbulos Renais/metabolismo , Metaloproteínas/metabolismo , Transporte Proteico/genética , Técnicas do Sistema de Duplo-Híbrido
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