FEATURES

FAMILY

DESCRIPTION

Also known as ABCD4_HUMAN, ABCD4, PXMP1L. Lysosomal membrane protein that transports cobalamin (Vitamin B12) from the lysosomal lumen to the cytosol in an ATP-dependent manner (PubMed:22922874, PubMed:33845046, PubMed:28572511, PubMed:31467407). Targeted by LMBRD1 lysosomal chaperone from the endoplasmic reticulum to the lysosomal membrane (PubMed:27456980). Then forms a complex with lysosomal chaperone LMBRD1 and cytosolic MMACHC to transport cobalamin across the lysosomal membrane (PubMed:25535791). Homodimer or heterodimer (PubMed:27456980, PubMed:33845046). Interacts with LMBRD1; this interaction induces the translocation of ABCD4 from the ER to the lysosome membrane (PubMed:27456980, PubMed:28572511). Interacts with LMBRD1 and MMACHC; this interaction ensures the transport of cobalamin from the lysosome to the cytosol (PubMed:25535791).

EXTERNAL LINKS

ABCD4

Lysosomal cobalamin transporter ABCD4

Structural Ligandability

Domains Chains

0 chains assessed

1 chain not associated with a domain

ABCD4

Structural Ligandability

Domains Chains

Distribution