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Mouse Anti-ABCD3 Recombinant Antibody (V2-6058) (CBMAB-0003CQ)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
V2-6058
Antibody Isotype
IgG1
Application
IF, IHC-P

Basic Information

Immunogen
Human PMP70 aa 410-474
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:1,000
IHC1:1,000
IF(ICC)1:100

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, pH 7.4, 50% Glycerol
Preservative
0.09% sodium azide
Concentration
Batch dependent
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
ATP-binding cassette, sub-family D (ALD), member 3
Introduction
The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. Mutation in ABCD3 have been found in two individuals affected by Zellweger syndrome. However, the role of ABCD3 in the causation of Zellweger syndrome remains uncertain. 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. Probable transporter involved in the transport of branched-chain fatty acids and C27 bile acids into the peroxisome; the latter function is a crucial step in bile acid biosynthesis. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.
Entrez Gene ID
5825
UniProt ID
P28288
Alternative Names
ATP-Binding Cassette, Sub-Family D (ALD), Member 3; 70 KDa Peroxisomal Membrane Protein; PXMP1; PMP70; DJ824O18.1 (ATP-Binding Cassette, Sub-Family D (ALD), Member 3 (PMP70, PXMP1)); Peroxisomal Membrane Protein 1 (70kD, Zellweger Syndrome); Peroxisomal Membrane Protein-1 (70kD); ABC43; CBAS5; ZWS2
Function
Probable transporter involved in the transport of branched-chain fatty acids and C27 bile acids into the peroxisome; the latter function is a crucial step in bile acid biosynthesis. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.
Biological Process
Fatty acid beta-oxidation
Fatty acid biosynthetic process
Lipid transport
Long-chain fatty acid import into peroxisome
Peroxisome organization
Protein import into peroxisome membrane
Response to drug
Response to organic cyclic compound
Very long-chain fatty acid catabolic process
Cellular Location
Peroxisome membrane
Involvement in disease
An autosomal recessive disorder characterized by hepatosplenomegaly, hepatic fibrosis, progressive liver failure, and accumulation of peroxisomal C27-bile acid intermediates in plasma.
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For research use only. Not intended for any clinical use.

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