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Rabbit Anti-ABCD4/BF594 Conjugated antibody (bs-11908R-BF594)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-11908R-BF594
英文名稱 Rabbit Anti-ABCD4/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的三磷酸腺苷結(jié)合盒轉(zhuǎn)運(yùn)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(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  轉(zhuǎn)運(yùn)蛋白  細(xì)胞膜蛋白  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,  (predicted: Mouse, Rat, )
產(chǎn)品應(yīng)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 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(chǔ) 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(chǎn)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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