Rabbit Polyclonal ABCD4 antibody. Suitable for WB and reacts with Human samples. Immunogen corresponding to Synthetic Peptide within Human ABCD4.
pH: 7.4
Preservative: 0.02% Sodium azide
Constituents: 50% Glycerol (glycerin, glycerine), 49% PBS, 0.88% Sodium chloride
WB | |
---|---|
Human | Tested |
Mouse | Predicted |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info 1/500.00000 - 1/1000.00000 | Notes - |
Species | Dilution info | Notes |
---|---|---|
Species Mouse | Dilution info - | Notes - |
Lysosomal membrane protein that transports cobalamin (Vitamin B12) from the lysosomal lumen to the cytosol in an ATP-dependent manner (PubMed:22922874, PubMed:28572511, PubMed:31467407, PubMed:33845046). 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).
PXMP1L, ABCD4, Lysosomal cobalamin transporter ABCD4, ATP-binding cassette sub-family D member 4, PMP70-related protein, Peroxisomal membrane protein 1-like, Peroxisomal membrane protein 69, P70R, PXMP1-L, PMP69
Rabbit Polyclonal ABCD4 antibody. Suitable for WB and reacts with Human samples. Immunogen corresponding to Synthetic Peptide within Human ABCD4.
pH: 7.4
Preservative: 0.02% Sodium azide
Constituents: 50% Glycerol (glycerin, glycerine), 49% PBS, 0.88% Sodium chloride
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ABCD4 or ATP-binding cassette sub-family D member 4 functions as a transport protein involved in intracellular lipid trafficking. It belongs to the ATP-binding cassette (ABC) family which utilizes ATP hydrolysis to transport various substrates across cellular membranes. The molecular weight of ABCD4 is approximately 75 kilodaltons. It most commonly expresses itself in human tissues such as the brain liver kidney and heart. These areas suggest that ABCD4 plays essential roles in these organs' lipid-related processes.
ABCD4 facilitates the transport of cobalamin (vitamin B12) from lysosomes to the cytoplasm. It operates as part of a multi-protein complex that includes LMBD1 and CBLIF. The complex collectively ensures cobalamin accessibility for cellular metabolic reactions. A proper transport function is critical for supporting the synthesis of DNA and maintaining normal hematopoiesis through vitamin B12 processing.
The protein facilitates integration into the cobalamin metabolism pathway and the peroxisomal biogenesis pathway. ABCD4 closely interacts with proteins such as LMBD1 in the former pathway. It helps import mediated transport which is integral for B12 availability affecting subsequent protein activity like methionine synthase and methylmalonyl-CoA mutase. These activities ensure vital cellular processes such as nucleotide biosynthesis and lipid metabolism proceed smoothly.
ABCD4 mutations link to inborn errors like methylmalonic aciduria and vitamin B12 deficiency anemia. These conditions arise from impaired cobalamin processing due to dysfunctional transport activity. In particular during B12 deficiency anemia ABCD4’s interaction with vitamin B12 transport proteins such as LMBD1 results in inadequate vitamin transport leading to disrupted hematopoietic function and metabolic imbalances.
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This species and application combination has not been tested, but we predict it will work based on strong homology. However, this combination is not covered by our product promise.
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All lanes: Western blot - Anti-ABCD4 antibody (ab135480) at 1/500 dilution
All lanes: Lysates from HeLa cells at 30 µg
Predicted band size: 69 kDa
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