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Code | CSB-PA000786 |
Size | US$167 |
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Have Questions? | Leave a Message or Start an on-line Chat |
Uniprot No. | O14678 | ||||||||
Target Names | ABCD4 | ||||||||
Alternative Names | ABCD4; PXMP1L; Lysosomal cobalamin transporter ABCD4; ATP-binding cassette sub-family D member 4; PMP70-related protein; P70R; Peroxisomal membrane protein 1-like; PXMP1-L; Peroxisomal membrane protein 69; PMP69 | ||||||||
Raised in | Rabbit | ||||||||
Species Reactivity | Human,Mouse | ||||||||
Immunogen | Synthesized peptide derived from the Internal region of Human ABCD4. | ||||||||
Immunogen Species | Homo sapiens (Human) | ||||||||
Conjugate | Non-conjugated | ||||||||
Isotype | IgG | ||||||||
Purification Method | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. | ||||||||
Concentration | It differs from different batches. Please contact us to confirm it. | ||||||||
Buffer | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. | ||||||||
Form | Liquid | ||||||||
Tested Applications | WB, IHC, ELISA | ||||||||
Recommended Dilution |
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Protocols | Western Blotting(WB) Protocol Immunohistochemistry (IHC) Protocol ELISA Protocol |
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Troubleshooting and FAQs | Antibody FAQs | ||||||||
Storage | Upon receipt, store at -20°C or -80°C. Avoid repeated freeze. | ||||||||
Lead Time | Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time. |
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Function |
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.
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Gene References into Functions |
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Involvement in disease | Methylmalonic aciduria and homocystinuria type cblJ (MAHCJ) |
Subcellular Location | Endoplasmic reticulum membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein. |
Protein Families | ABC transporter superfamily, ABCD family, Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily |
Tissue Specificity | Ubiquitous. |
Database Links |
HGNC: 68 OMIM: 603214 KEGG: hsa:5826 STRING: 9606.ENSP00000349396 UniGene: Hs.94395 |