Mouse anti-Human ABCD3 Monoclonal Antibody | anti-ABCD3 antibody

ABCD3 (ATP-binding Cassette Sub-family D Member 3, 70kD Peroxisomal Membrane Protein, PMP70, PXMP1) (MaxLight 650)

Cat. Num. AAA6220702

• Gene Names: ABCD3; ZWS2; ABC43; CBAS5; PMP70; PXMP1
• Reactivity: Human
• Applications: FLISA
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ABCD3; Monoclonal Antibody; ABCD3 (ATP-binding Cassette Sub-family D Member 3; 70kD Peroxisomal Membrane Protein; PMP70; PXMP1) (MaxLight 650); anti-ABCD3 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 2H6
• Specificity: Recognizes human ABCD3.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight650.

• Applicable Applications for anti-ABCD3 antibody: FLISA
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa351-450, from human ABCD3 (NP_002849) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: SELLEDYYQSGRMLLRMSQALGRIVLAGREMTRLAGFTARITELMQVLKDLNHGKYERTMVSQQEKGIEGVQVIPLIPGAGEIIIADNIIKFDHVPLAT
• Conjugate: MaxLight650
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight650 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Related Product Information for anti-ABCD3 antibody

Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.

Product Categories/Family for anti-ABCD3 antibody

Antibodies; Multi Drug Resistance (MDR)

NCBI and Uniprot Product Information

• NCBI GI #: 4506341
• NCBI GeneID: 5825
• NCBI Accession #: NP_002849
• NCBI GenBank Nucleotide #: NM_002858
• UniProt Accession #: P28288
• Molecular Weight: 75kDa
• NCBI Official Full Name: ATP-binding cassette sub-family D member 3 isoform a
• NCBI Official 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
• Protein Family: ABC transporter D family
• UniProt Gene Name: ABCD3
• UniProt Synonym Gene Names: PMP70; PXMP1; PMP70
• 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: 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