ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells)
ATP + H2O + RCbl → ADP + Pi + RCbl
- Reactome
- R-HSA-5223313
- Compartment
- cytosol · lysosomal lumen · lysosomal membrane
- Rhea
- RHEA:17873
Inputs
- ATPCHEBI:30616, C10H12N5O13P3, cytosol
- H2OCHEBI:15377, H2O, cytosol
- RCblCHEBI:140785, C62H88CoN13O14PR, lysosomal lumen
Outputs
- ADPCHEBI:456216, C10H12N5O10P2, cytosol
- PiCHEBI:43474, HO4P, cytosol
- RCblCHEBI:140785, C62H88CoN13O14PR, cytosol
Catalysed by
- ABC-type vitamin B12 transporter activity of ABCD4:LMBRD1 [lysosomal membrane]EC 7.6.2.8
Literature
- Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism Nat. Genet., 2012
- Translocation of the ABC transporter ABCD4 from the endoplasmic reticulum to lysosomes requires the escort protein LMBD1 Sci Rep, 2016
- The lysosomal protein ABCD4 can transport vitamin B 12 across liposomal membranes in vitro J Biol Chem, 2021
- Identification of a putative lysosomal cobalamin exporter altered in the cblF defect of vitamin B12 metabolism Nat. Genet., 2009
- Defect in vitamin B12 release from lysosomes: newly described inborn error of vitamin B12 metabolism Science, 1985