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1.
Traffic ; 13(1): 108-19, 2012 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-21985295

RESUMEN

Chediak-Higashi syndrome is an autosomal recessive disorder that affects vesicle morphology. The Chs1/Lyst protein is a member of the BEige And CHediak family of proteins. The absence of Chs1/Lyst gives rise to enlarged lysosomes. Lysosome size is regulated by a balance between vesicle fusion and fission and can be reversibly altered by acidifying the cytoplasm using Acetate Ringer's or by incubating with the drug vacuolin-1. We took advantage of these procedures to determine rates of lysosome fusion and fission in the presence or absence of Chs1/Lyst. Here, we show by microscopy, flow cytometry and in vitro fusion that the absence of the Chs1/Lyst protein does not increase the rate of lysosome fusion. Rather, our data indicate that loss of this protein decreases the rate of lysosome fission. We further show that overexpression of the Chs1/Lyst protein gives rise to a faster rate of lysosome fission. These results indicate that Chs1/Lyst regulates lysosome size by affecting fission.


Asunto(s)
Síndrome de Chediak-Higashi , Lisosomas/ultraestructura , Macrófagos/ultraestructura , Proteínas/metabolismo , Proteínas de Transporte Vesicular/metabolismo , Animales , Células Cultivadas , Síndrome de Chediak-Higashi/metabolismo , Síndrome de Chediak-Higashi/patología , Fibroblastos/efectos de los fármacos , Fibroblastos/metabolismo , Fibroblastos/ultraestructura , Citometría de Flujo , Compuestos Heterocíclicos de 4 o más Anillos/farmacología , Humanos , Péptidos y Proteínas de Señalización Intracelular , Lisosomas/efectos de los fármacos , Lisosomas/metabolismo , Macrófagos/efectos de los fármacos , Macrófagos/metabolismo , Ratones , Ratones Endogámicos C57BL , Proteínas/genética , Proteínas de Transporte Vesicular/genética
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