Lysosomal cobalamin transporter ABCD4

Definition:

Lysosomal membrane protein that transports cobalamin (Vitamin B12) from the lysosomal lumen to the cytosol in an ATP-dependent manner. Targeted by LMBRD1 lysosomal chaperone from the endoplasmic reticulum to the lysosomal membrane. Then forms a complex with lysosomal chaperone LMBRD1 and cytosolic MMACHC to transport cobalamin across the lysosomal membrane.

References:

[1]. Da Xu, et al. Cryo-EM structure of human lysosomal cobalamin exporter ABCD4. Cell Res. 2019 Dec;29(12):1039-1041. [Content Brief]

[2]. David Coelho, et al. Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism. Nat Genet. 2012 Oct;44(10):1152-5. [Content Brief]

[3]. Katsuki Kitai, et al. The lysosomal protein ABCD4 can transport vitamin B12 across liposomal membranes in vitro. J Biol Chem. 2021 Jan-Jun;296:100654. [Content Brief]

[4]. Justin C Deme, et al. Purification and interaction analyses of two human lysosomal vitamin B12 transporters: LMBD1 and ABCD4. Mol Membr Biol. 2014 Nov-Dec;31(7-8):250-61. [Content Brief]

[5]. Kosuke Kawaguchi, et al. Translocation of the ABC transporter ABCD4 from the endoplasmic reticulum to lysosomes requires the escort protein LMBD1. Sci Rep. 2016 Jul 26;6:30183. [Content Brief]

[6]. Victoria Fettelschoss, et al. Clinical or ATPase domain mutations in ABCD4 disrupt the interaction between the vitamin B12-trafficking proteins ABCD4 and LMBD1. J Biol Chem. 2017 Jul 14;292(28):11980-11991. [Content Brief]

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