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Rabbit Anti-PXMP1/PMP70/BF594 Conjugated antibody (bs-8584R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-8584R-BF594
英文名稱 Rabbit Anti-PXMP1/PMP70/BF594 Conjugated antibody
中文名稱 BF594標記的過氧化物酶膜蛋白1抗體
別    名 70 kDa peroxisomal membrane protein; ABC 43; ABC D3; ABC43; ABCD 3; ABCD3; 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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
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
交叉反應
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 75kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PXMP1/ABCD3
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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