ABCD3 Recombinant Monoclonal Antibody

Code CSB-RA843022A0HU
Size US$210
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Image
  • Western Blot
    Positive WB detected in:Mouse brain tissue lysate(30µg), Rat brain tissue lysate(30µg)
    All lanes: ABCD3 antibody at 1:1000
    Secondary
    Goat polyclonal to rabbit IgG at 1/40000 dilution
    Predicted band size: 75 kDa
    Observed band size:70 kDa
    Exposure time:30s
  • IHC image of CSB-RA843022A0HU diluted at 1:100 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
  • Overlay Peak curve showing HepG2 cells stained with CSB-RA843022A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
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Product Details

Uniprot No.
Target Names
ABCD3
Alternative Names
ATP-binding cassette sub-family D member 3 (70 kDa peroxisomal membrane protein) (PMP70), ABCD3, PMP70 PXMP1
Species Reactivity
Human, Mouse, Rat
Immunogen
A synthesized peptide derived from Human ABCD3 protein
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Clonality
Monoclonal
Isotype
Rabbit IgG
Clone No.
16H5
Purification Method
Affinity-chromatography
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
Form
Liquid
Tested Applications
ELISA, WB, IHC, FC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:50-1:200
FC 1:20-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
Broad substrate specificity ATP-dependent transporter of the ATP-binding cassette (ABC) family that catalyzes the transport of long-chain fatty acids (LCFA)-CoA, dicarboxylic acids-CoA, long-branched-chain fatty acids-CoA and bile acids from the cytosol to the peroxisome lumen for beta-oxydation. Has fatty acyl-CoA thioesterase and ATPase activities. Probably hydrolyzes fatty acyl-CoAs into free fatty acids prior to their ATP-dependent transport into peroxisomes. Thus, play a role in regulation of LCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation.
Gene References into Functions
  1. ABCD1 and ABCD2 are involved in the transport of long and very long chain fatty acids (VLCFA) or their CoA-derivatives into peroxisomes with different substrate specificities, while ABCD3 is involved in the transport of branched chain acyl-CoA into peroxisomes.ABCD4 is deduced to take part in the transport of vitamin B12 from lysosomes into the cytosol. PMID: 27766264
  2. Data show that ACBD3 can recruit PI4KB to model membranes as well as redirect PI4KB to cellular membranes where it is not naturally found. Also, results show that ACBD3 regulates the enzymatic activity of PI4KB kinase through membrane recruitment rather than allostery. PMID: 27009356
  3. The N-terminal motif of PMP70 suppresses cotranslational targeting to the endoplasmic reticulum. PMID: 26711236
  4. Increased ABCD3 expression correlates with Gleason Score. PMID: 25802834
  5. Deficiency of peroxisomal ABCD3 resulted in bile acid biosynthesis defect. PMID: 25168382
  6. We postulate a role for human ABCD3 in the oxidation of dicarboxylic acids and a role in buffering fatty acids that are overflowing from the mitochondrial beta-oxidation system PMID: 24333844
  7. investigation of organelle-targeting properties of N-terminal portions of peroxisomal PMP70; amino acid sequence and domain structure of human form discussed PMID: 20007743
  8. Testosterone metabolites did not alter expression of ABCD3 mRNA in fibroblasts from X-linked adrenoleukodystrophy patients. PMID: 17602313
  9. ALDRP interacts with PMP70. This interaction occurs via the ALDRP C-terminus [374-740] and the PMP70 C-terminus [338-659]. This interaction was demonstrated using human PMP70 and mouse ALDRP. PMID: 10551832
  10. ALDP interacts with PMP70. This interaction occurs via the C-terminus of ALDP [361-745] and the C-terminus of PMP70 [338-659]. ALDP mutations P484R and R591Q abolish the interaction. PMID: 10551832
  11. PMP70 (ABCD3) homodimerizes via the carboxy terminal half [338-659]. PMID: 10551832

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Involvement in disease
Congenital bile acid synthesis defect 5 (CBAS5)
Subcellular Location
Peroxisome membrane; Multi-pass membrane protein.
Protein Families
ABC transporter superfamily, ABCD family, Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily
Database Links

HGNC: 67

OMIM: 170995

KEGG: hsa:5825

STRING: 9606.ENSP00000359233

UniGene: Hs.700576

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