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| Code | CSB-PA039868 |
| Size | US$299 |
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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 | Synthetic peptide of Human ABCD4 | ||||||
| Immunogen Species | Homo sapiens (Human) | ||||||
| Conjugate | Non-conjugated | ||||||
| Isotype | IgG | ||||||
| Purification Method | Antigen affinity purification | ||||||
| Concentration | It differs from different batches. Please contact us to confirm it. | ||||||
| Buffer | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol | ||||||
| Form | Liquid | ||||||
| Tested Applications | ELISA,WB | ||||||
| Recommended Dilution |
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| Protocols | ELISA Protocol Western Blotting(WB) 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 |
