期刊
TRAFFIC
卷 13, 期 8, 页码 1160-1169出版社
WILEY-BLACKWELL
DOI: 10.1111/j.1600-0854.2012.01375.x
关键词
BLOC-1; BLOS1; Hermansky-Pudlak syndrome; KXD1; lysosome-related organelles
类别
资金
- National Natural Science Foundation of China [31071252, 81101182, 30730049]
- Ministry of Agriculture of PRC [2009ZX08009-158B]
- Chinese Academy of Sciences [KSCX2-EW-R-05]
- National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA
Biogenesis of lysosome-related organelles (LROs) complex-1 (BLOC-1) is an eight-subunit complex involved in lysosomal trafficking. Interacting proteins of these subunits expand the understanding of its biological functions. With the implementation of the naive Bayesian analysis, we found that a human uncharacterized 20 kDa coiled-coil KxDL protein, KXD1, is a BLOS1-interacting protein. In vitro binding assays confirmed the interaction between BLOS1 and KXD1. The mouse KXD1 homolog was widely expressed and absent in Kxd1 knockout (KO) mice. BLOS1 was apparently reduced in Kxd1-KO mice. Mild defects in the melanosomes of the retinal pigment epithelia and in the platelet dense granules of the Kxd1-KO mouse were observed, mimicking a mouse model of mild HermanskyPudlak syndrome that affects the biogenesis of LROs.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据