Cell Biology Antibodies 14

Anti-ABCD3 Antibody (CAB18177)

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SKU:
CAB18177
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-ABCD3 Antibody
Antibody SKU:CAB18177
Antibody Size:20uL, 50uL, 100uL
Application:WB IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant protein of human ABCD3.
Application:WB IF
Recommended Dilution:WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:HeLa, HepG2, Mouse liver, Mouse kidney, Rat liver
Immunogen:Recombinant protein of human ABCD3.
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:5825
Uniprot:P28288
Cellular Location:
Calculated MW:
Observed MW:70KDa
Anti-ABCD3 Antibody (CAB18177)Western blot - ABCD3 Rabbit pAb (CAB18177)
Synonyms:ABC43, CBAS5, PMP70, PXMP1, ZWS2, ABCD3
Background: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 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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