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Mucolipin-1 is a lysosomal membrane protein required for intracellular lactosylceramide traffic

Pryor, P.R., Reimann, F., Gribble, F.M. and Luzio, J.P. (2006) Mucolipin-1 is a lysosomal membrane protein required for intracellular lactosylceramide traffic. Traffic, 7 (10). pp. 1388-1398. ISSN 1398-9219

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Abstract

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.

Item Type: Article
Institution: The University of York
Academic Units: The University of York > Biology (York)
Depositing User: York RAE Import
Date Deposited: 10 Feb 2009 19:06
Last Modified: 10 Feb 2009 19:07
Published Version: http://dx.doi.org/10.1111/j.1600-0854.2006.00475.x
Status: Published
Publisher: Blackwell Publishing
Identification Number: 10.1111/j.1600-0854.2006.00475.x
URI: http://eprints.whiterose.ac.uk/id/eprint/7782

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