Rabbit anti-Human ABCD3 Polyclonal Antibody | anti-ABCD3 antibody

Anti-ABCD3 Antibody

Cat. Num. AAA416812

• Gene Names: ABCD3; ZWS2; ABC43; PMP70; PXMP1
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: Affinity purified
• Synonyms: ABCD3; Polyclonal Antibody; Anti-ABCD3 Antibody; anti-ABCD3 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Affinity purified
• Form/Format: Liquid
• Concentration: 100ug/100ul (varies by lot)
• Sequence Length: 236

• Applicable Applications for anti-ABCD3 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Optimal dilutions to be determined by researchers
• Storage Buffer: PBS, pH 7.4 with 0.02% Sodium Azide
• Immunogen: Recombinant protein
• Preparation and Storage: This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Testing Data

Related Product Information for anti-ABCD3 antibody

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]

NCBI and Uniprot Product Information

• NCBI GI #: 169881286
• NCBI GeneID: 5825
• NCBI Accession #: NP_001116146.1
• NCBI GenBank Nucleotide #: NM_001122674.1
• Molecular Weight: 27,121 Da
• NCBI Official Full Name: ATP-binding cassette sub-family D member 3 isoform b
• NCBI Official Synonym Full Names: ATP-binding cassette, sub-family D (ALD), member 3
• NCBI Official Symbol: ABCD3
• NCBI Official Synonym Symbols: ZWS2; ABC43; PMP70; PXMP1
• NCBI Protein Information: ATP-binding cassette sub-family D member 3; 70 kDa peroxisomal membrane protein; Peroxisomal membrane protein-1 (70kD); dJ824O18.1 (ATP-binding cassette, sub-family D (ALD), member 3 (PMP70, PXMP1)); peroxisomal membrane protein 1 (70kD, Zellweger syndrome)
• UniProt Protein Name: ATP-binding cassette sub-family D member 3
• Protein Family: ABC transporter D family
• UniProt Gene Name: ABCD3
• UniProt Entry Name: ABCD3_HUMAN

NCBI Description

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 Description

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.

Protein type: Mitochondrial; Transporter, ABC family; Membrane protein, integral; Membrane protein, multi-pass; Transporter

Chromosomal Location of Human Ortholog: 1p21.3

Cellular Component: peroxisomal membrane; peroxisomal matrix; intracellular membrane-bound organelle; membrane; 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; peroxisome organization and biogenesis; very-long-chain fatty acid catabolic process; transmembrane transport

Disease: Bile Acid Synthesis Defect, Congenital, 5