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ABCD4 Rabbit pAb (bs-11908R)  
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產品編號 bs-11908R
英文名稱 ABCD4 Rabbit pAb
中文名稱 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.  
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
克 隆 號
交叉反應 Human (predicted: Mouse,Rat)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 69 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 ABCD4: 351-450/606 
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.

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.

SWISS:
O14678

Gene ID:
5826

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



產品圖片
Sample: A431 Cell (Human) Lysate at 30 ug Primary: Anti- ABCD4 (bs-11908R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 69 kD Observed band size: 69 kD
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