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Product Name ABCD3 Chinese Name 过氧化物酶膜蛋白1抗体 Alias 70 kDa peroxisomal membrane protein; ABC 43; ABC D3; ABC43; ABCD 3; ABCD3 protein; ABCD3_HUMAN; ATP binding cassette sub family D (ALD) member 3; ATP binding cassette sub family D member 3; ATP-binding cassette sub-family D member 3; Peroxisomal membrane protein 1; PMP 70; PMP70; PXMP 1; PXMP1; ZWS2. Research Area Tumour Cell biology immunology Neurobiology Alzheimer's Immunogen Species Rabbit Clonality Polyclonal React Species Human, (predicted: Mouse, Rat, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 75kDa Cellular localization cytoplasmic The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human PXMP1/ABCD3: 51-160/659 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail The peroxisomal membrane contains several ATP-binding cassette (ABC) transporters, ABCD1-4 that are known to be present in the human peroxisome membrane. All four proteins are ABC half-transporters, which dimerize to form an active transporter. A mutation in the ABCD1 gene causes X-linked adreno-leukodystrophy (X-ALD), a peroxisomal disorder which affects lipid storage. ABCD2 in mouse is expressed at high levels in the brain and adrenal organs, which are adversely affected in X-ALD. The peroxisomal membrane comprises two quantitatively major proteins, PMP22 and ABCD3. ABCD3 is associated with irregularly shaped vesicles which may be defective peroxisomes or peroxisome precursors. ABCD1 localizes to peroxisomes. ABCB7 is a half-transporter involved in the transport of heme from the mitochondria to the cytosol.
Function:
Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.
Subunit:
Can form heterodimers with ABCD1/ALD and ABCD2/ALDR. Dimerization is necessary to form an active transporter. Interacts with PEX19.
Subcellular Location:
Peroxisome membrane; Multi-pass membrane protein.
Similarity:
Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily.
Contains 1 ABC transmembrane type-1 domain.
Contains 1 ABC transporter domain.
SWISS:
P28288
Gene ID:
5825
Database links:Entrez Gene: 100765314 Hamster
Entrez Gene: 5825 Human
Entrez Gene: 19299 Mouse
Omim: 170995 Human
SwissProt: P28288 Human
SwissProt: P55096 Mouse
Unigene: 700576 Human
Unigene: 399042 Mouse
Unigene: 7024 Rat
Product Picture Tissue/cell: human cervical carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-PXMP1/ABCD3/PMP70 Polyclonal Antibody, Unconjugated(SL8584R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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