Cloned (Comment) | Organism |
---|---|
gene ABCD4, recombinant expression of His-tagged protein ABCD4 in Pichia pastoris strain SMD1168 | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
AlF3 | inhibition of the ATPase activity of liposome-reconstituted His-hABCD4 | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
lysosomal membrane | ABCD4 dimer makes a complex with lysosomal membrane protein LMBD1, an adopter protein, and targets to lysosome | Homo sapiens | 5765 | - |
additional information | ABCD4 barely interacts with the peroxisomal biogenesis factor Pex19p, because of its lack of NH2-terminal hydrophobic motif. As a result, the transmembrane domain (TMD) 1 of ABCD4 is recognized by certain signal recognition particles and integrated into the ER membrane. ABCD4 dimer then makes a complex with lysosomal membrane protein LMBD1, an adopter protein, and targets to lysosome | Homo sapiens | - |
- |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Mg2+ | required | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + H2O + vitamin B12-[cobalamin-binding protein][side 1] | Homo sapiens | - |
ADP + phosphate + vitamin B12[side 2] + [cobalamin-binding protein][side 1] | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | O14678 | - |
- |
Purification (Comment) | Organism |
---|---|
recombinant His-tagged protein ABCD4 from Pichia pastoris strain SMD1168 by detergent solubilization and nickel affinity chromatography, evaluation of diverse detergents | Homo sapiens |
Renatured (Comment) | Organism |
---|---|
recombinant His-tagged protein ABCD4 protein solubilization and reconstitution in proteoliposomes | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + H2O + vitamin B12-[cobalamin-binding protein][side 1] | - |
Homo sapiens | ADP + phosphate + vitamin B12[side 2] + [cobalamin-binding protein][side 1] | - |
? | |
additional information | the enzyme possesses equal levels of acyl-CoA thioesterase activity, ATPase and thioesterase activities of liposome-reconstituted human ABCD4, overview | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
ABCD4 | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
7.6 | - |
assay at | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
ATP | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | ABCD4 dysfunction results in a failure of lysosomal vitamin B12 release | Homo sapiens |
physiological function | ABCD4 is located on lysosomal membrane and is involved in the transport of vitamin B12 from lysosomes to the cytosol | Homo sapiens |