Anti-PMP70/ABCD3 Antibody (CAB4172)
- SKU:
- CAB4172
- Product Type:
- Antibody
- Antibody Type:
- Monoclonal Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- ABC43
- Synonyms:
- CBAS5
- Synonyms:
- PMP70
- Synonyms:
- PXMP1
- Synonyms:
- ZWS2
- Research Area:
- Cell Biology
Description
| Product Name: | PMP70/ABCD3 Rabbit mAb |
| Product Code: | CAB4172 |
| Size: | 20uL, 50uL, 100uL |
| Synonyms: | ABC43, CBAS5, PMP70, PXMP1, ZWS2 |
| Applications: | WB, IF |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | A synthesized peptide derived from human PMP70/ABCD3 |
| Applications: | WB, IF |
| Recommended Dilutions: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | HepG2, A-549, Mouse liver, Mouse kidney, Rat liver |
| Immunogen: | A synthesized peptide derived from human PMP70/ABCD3 |
| Purification Method: | Affinity purification |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | Email for sequence |
| Gene ID: | 5825 |
| Uniprot: | P28288 |
| Calculated MW: | 70kDa |
| Observed MW: | 70KDa |
| UniProt Protein Function: | ABCD3: a member of the superfamily of ATP-binding cassette (ABC) transporters. Likely plays an important role in peroxisome biogenesis. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ALD subfamily, which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. Defects in ABCD3 may be the cause of Zellweger syndrome-2 (ZWS-2), an autosomal recessive disorder due to defective import mechanisms for peroxisomal matrix enzymes. |
| UniProt Protein Details: | Protein type:Membrane protein, multi-pass; Transporter, ABC family; Transporter; Membrane protein, integral; Mitochondrial Chromosomal Location of Human Ortholog: 1p21.3 Cellular Component: peroxisomal membrane; peroxisomal matrix; membrane; intracellular membrane-bound organelle; mitochondrial inner membrane; integral to membrane; peroxisome; cytosol Molecular Function:protein binding; protein homodimerization activity; ATPase activity, coupled to transmembrane movement of substances; ATPase activity; ATP binding Biological Process: fatty acid beta-oxidation; very-long-chain fatty acid catabolic process; peroxisome organization and biogenesis; transmembrane transport Disease: Bile Acid Synthesis Defect, Congenital, 5 |
| NCBI Summary: | The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ALD subfamily, which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. This peroxisomal membrane protein likely plays an important role in peroxisome biogenesis. Mutations have been associated with some forms of Zellweger syndrome, a heterogeneous group of peroxisome assembly disorders. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P28288 |
| NCBI GenInfo Identifier: | 130358 |
| NCBI Gene ID: | 5825 |
| NCBI Accession: | P28288.1 |
| UniProt Related Accession: | P28288 |
| Molecular Weight: | 70kDa |
| NCBI Full Name: | ATP-binding cassette sub-family D member 3 |
| NCBI Synonym Full Names: | ATP binding cassette subfamily D member 3 |
| NCBI Official Symbol: | ABCD3 |
| NCBI Official Synonym Symbols: | ZWS2; ABC43; CBAS5; PMP70; PXMP1 |
| NCBI Protein Information: | ATP-binding cassette sub-family D member 3 |
| UniProt Protein Name: | ATP-binding cassette sub-family D member 3 |
| UniProt Synonym Protein Names: | 70 kDa peroxisomal membrane protein; PMP70 |
| UniProt Gene Name: | ABCD3 |
| UniProt Entry Name: | ABCD3_HUMAN |
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