ABCD3 Rabbit pAb
- 产品详情
- 实验流程
Application
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | P28288 |
| Other Accession | P28288 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 75476 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human PXMP1/ABCD3 |
| Epitope Specificity | 51-160/659 |
| 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 | Can form heterodimers with ABCD1/ALD and ABCD2/ALDR. Dimerization is necessary to form an active transporter. Interacts with PEX19. |
| 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 | 5825 |
|---|---|
| Other Names | I79_023932; ABC43; CBAS5; PMP70; PXMP1; ZWS2; PMP68; ABCD3_HUMAN; ABCD3; 70 kDa peroxisomal membrane protein (PMP70); 3.1.2.-; 7.6.2.-; ABCD3_MOUSE; 68 kDa peroxisomal membrane protein (PMP68); ABCD3_RAT; ATP binding cassette subfamily D member 3; ATP-binding cassette, sub-family D (ALD), member 3; peroxisomal membrane protein 1 (70kD, Zellweger syndrome); peroxisomal membrane protein 70 kDa |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| 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 | ABCD3 {ECO:0000303|PubMed:39223112, ECO:0000312|HGNC:HGNC:67} |
|---|---|
| Function | ATP-dependent transporter of the ATP-binding cassette (ABC) family that catalyzes the transport of long- and very-long-chain fatty acyl-CoAs and probably bile acyl-CoAs from the cytosol to the peroxisome matrix for beta-oxydation. Appears to be specifically involved in the peroxisomal transport of branched-chain fatty acyl-CoAs (BCFA-CoAs) and dicarboxylic acyl CoAs (DCA-CoAs) (PubMed:11248239, PubMed:23671276, PubMed:24333844, PubMed:25168382, PubMed:29397936, PubMed:39223112, PubMed:41428872). Has fatty acyl-CoA thioesterase and ATPase activities. Probably hydrolyzes fatty acyl-CoAs into free fatty acids prior to their ATP-dependent transport into peroxisomes. |
| Cellular Location | Peroxisome membrane; Multi-pass membrane protein |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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