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ABCD3 Rabbit pAb (bs-8584R)  
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產品編號 bs-8584R
英文名稱 ABCD3 Rabbit pAb
中文名稱 過氧化物酶膜蛋白1抗體
別    名 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.  
研究領域 腫瘤  細胞生物  免疫學  神經生物學  Alzheimer's  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human (predicted: Mouse,Rat)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 75 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PXMP1/ABCD3: 51-160/659 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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

Entrez Gene: 25270 Rat

Omim: 170995 Human

SwissProt: P28288 Human

SwissProt: P55096 Mouse

SwissProt: P16970 Rat

Unigene: 700576 Human

Unigene: 399042 Mouse

Unigene: 7024 Rat



產品圖片
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(bs-8584R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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