Reaction: ABCA5 transports CHOL from lysosomal lumen to cytosol
- in pathway: ABC transporters in lipid homeostasis
The ABC transporter subfamily A member ABCA5 is thought to mediate cholesterol (CHOL) efflux from lysosomes (Petry et al. 2003). ABCA5 defects are associated with autosomal recessive congenital generalised hypertrichosis terminalis (CGHT; MIM:135400). Lysosomal function is reduced with an accumulation of autophagosomes and their cargo as well as increased endolysosomal cholesterol in CGHT keratinocytes, resulting in hair overgrowth (DeStefano et al. 2014).
Reaction - small molecule participants:
Pi [cytosol]
CHOL [cytosol]
ADP [cytosol]
H2O [cytosol]
ATP [cytosol]
CHOL [lysosomal lumen]
Reactome.org reaction link: R-HSA-8848053
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Reaction input - small molecules:
water
ATP(4-)
cholesterol
Reaction output - small molecules:
hydrogenphosphate
cholesterol
ADP(3-)
Reactome.org link: R-HSA-8848053