Mucolipin-1 is a lysosomal membrane protein required for intracellular lactosylceramide traffic.
Traffic
; 7(10): 1388-98, 2006 Oct.
Article
em En
| MEDLINE
| ID: mdl-16978393
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Antígenos CD
/
Endocitose
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Canais de Cátion TRPM
/
Lactosilceramidas
/
Lisossomos
Limite:
Animals
/
Humans
Idioma:
En
Revista:
Traffic
Assunto da revista:
FISIOLOGIA
Ano de publicação:
2006
Tipo de documento:
Article
País de afiliação:
Reino Unido