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Rabbit Anti-ABCD4/HRP Conjugated antibody (bs-11908R-HRP)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11908R-HRP
英文名稱 Rabbit Anti-ABCD4/HRP Conjugated antibody
中文名稱 辣根過氧化物酶標記的三磷酸腺苷結合盒轉運蛋白4抗體
別    名 ABC 41; ABC41; ABCD 4; ABCD4; ABCD4_HUMAN; ATP binding cassette sub family D (ALD) member 4; ATP binding cassette sub family D member 4; ATP-binding cassette sub-family D member 4; P70R antibodyP79R; Peroxisomal membrane protein 1 like; Peroxisomal membrane protein 1-like; Peroxisomal membrane protein 69; PMP 69; PMP69; PMP70-related protein; XMP 1L; PXMP1 L; PXMP1-L; PXMP1L.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  信號轉導  轉運蛋白  細胞膜蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,  (predicted: Mouse, Rat, )
產品應用 WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 69kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ABCD4
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.

Subunit:
Homodimer or heterodimer

Subcellular Location:
Peroxisome membrane; Multi-pass membrane protein.

Tissue Specificity:
Ubiquitous.

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: 453032 Chimpanzee

Entrez Gene: 490781 Dog

Entrez Gene: 5826 Human

Entrez Gene: 19300 Mouse

Entrez Gene: 299196 Rat

GenBank: NM_005050 Human

Omim: 603214 Human

SwissProt: O14678 Human

SwissProt: O89016 Mouse



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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