期刊
TRAFFIC
卷 7, 期 10, 页码 1388-1398出版社
WILEY
DOI: 10.1111/j.1600-0854.2006.00475.x
关键词
endocytosis; lysosomes; organelle fusion; transient receptor potential
类别
资金
- Medical Research Council [G9310915] Funding Source: researchfish
- Medical Research Council [G9310915] Funding Source: Medline
- Wellcome Trust [066438, 071187] Funding Source: Medline
- MRC [G9310915] Funding Source: UKRI
Mucolipin-1 is a membrane protein encoded by the gene MCOLN1, mutations in which result in the lysosomal storage disorder mucolipidosis type IV (MLIV). Efficient lysosomal targeting of mucolipin-1 requires di-leucine motifs in both the N-terminal and the C-terminal cytosolic tails. We have shown that aberrant lactosylceramide trafficking in MLIV cells may be rescued by wild-type mucolipin-1 expression but not by mucolipin-1 mistargeted to the plasma membrane or by lysosome-localized mucolipin-1 mutated in its predicted ion pore-selectivity region. Our data demonstrate that the correct localization of mucolipin-1 and the integrity of its ion pore are essential for its physiological function in the late endocytic pathway.
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