ABCD4 Rabbit pAb
- 产品详情
- 实验流程
Application
| WB |
|---|---|
| Primary Accession | O14678 |
| Other Accession | O14678 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 68597 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human ABCD4 |
| Epitope Specificity | 351-450/606 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily. Contains 1 ABC transmembrane type-1 domain. Contains 1 ABC transporter domain. |
| SUBUNIT | Homodimer or heterodimer |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | The peroxisomal membrane contains several ATP-binding cassette (ABC) transporters, ABCD1-4 that are known to be present in the human peroxisome membrane. All four proteins are ABC half-transporters, which dimerize to form an active transporter. A mutation in the ABCD1 gene causes X-linked adreno-leukodystrophy (X-ALD), a peroxisomal disorder which affects lipid storage. ABCD2 in mouse is expressed at high levels in the brain and adrenal organs, which are adversely affected in X-ALD. The peroxisomal membrane comprises two quantitatively major proteins, PMP22 and ABCD3. ABCD3 is associated with irregularly shaped vesicles which may be defective peroxisomes or peroxisome precursors. ABCD1 localizes to peroxisomes. ABCB7 is a half-transporter involved in the transport of heme from the mitochondria to the cytosol. |
| Gene ID | 5826 |
|---|---|
| Other Names | CK820_G0002881; ABC41; EST352188; MAHCJ; P70R; P79R; PMP69; PXMP1L; P69r; ABCD4_HUMAN; 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); 7.6.2.8; ABCD4_MOUSE; ATP binding cassette subfamily D member 4; ATP-binding cassette, sub-family D (ALD), member 4; Lysosomal cobalamin transporter ABCD4 |
| Dilution | WB=1:500-2000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | ABCD4 (HGNC:68) |
|---|---|
| Synonyms | PXMP1L |
| Function | 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). |
| Cellular Location | Endoplasmic reticulum membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein. Note=Targeted by LMBRD1 lysosomal chaperone to the lysosomal membrane. |
| Tissue Location | Ubiquitous. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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