重组人ABCD1/ALD蛋白(Active) (ab235011)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Active: Yes
- Tags: His tag C-Terminus
- Suitable for: Functional Studies, SDS-PAGE
描述
-
产品名称
重组人ABCD1/ALD蛋白(Active) -
生物活性
Measured by its binding ability in a functional ELISA. Immobilized ab235011 at 5 μg/ml can bind human PEX19, the EC50 of human PEX19 protein is 22.96-33.00 μg/ml.
-
纯度
> 90 % SDS-PAGE. -
表达系统
Escherichia coli -
Accession
-
蛋白长度
Full length protein -
无动物成分
No -
性质
Recombinant -
-
种属
Human -
序列
MPVLSRPRPWRGNTLKRTAVLLALAAYGAHKVYPLVRQCLAPARGLQAPA GEPTQEASGVAAAKAGMNRVFLQRLLWLLRLLFPRVLCRETGLLALHSAA LVSRTFLSVYVARLDGRLARCIVRKDPRAFGWQLLQWLLIALPATFVNSA IRYLEGQLALSFRSRLVAHAYRLYFSQQTYYRVSNMDGRLRNPDQSLTED VVAFAASVAHLYSNLTKPLLDVAVTSYTLLRAARSRGAGTAWPSAIAGLV VFLTANVLRAFSPKFGELVAEEARRKGELRYMHSRVVANSEEIAFYGGHE VELALLQRSYQDLASQINLILLERLWYVMLEQFLMKYVWSASGLLMVAVP IITATGYSESDAEAVKKAALEKKEEELVSERTEAFTIARNLLTAAADAIE RIMSSYKEVTELAGYTARVHEMFQVFEDVQRCHFKRPRELEDAQAGSGTI GRSGVRVEGPLKIRGQVVDVEQGIICENIPIVTPSGEVVVASLNIRVEEG MHLLITGPNGCGKSSLFRILGGLWPTYGGVLYKPPPQRMFYIPQRPYMSV GSLRDQVIYPDSVEDMQRKGYSEQDLEAILDVVHLHHILQREGGWEAMCD WKDVLSGGEKQRIGMARMFYHRPKYALLDECTSAVSIDVEGKIFQAAKDA GIALLSITHRPSLWKYHTHLLQFDGEGGWKFEKLDSAARLSLTEEKQRLE QQLAGIPKMQRRLQELCQILGEAVAPAHVPAPSPQGPGGLQGAST -
预测分子量
85 kDa including tags -
氨基酸
1 to 745 -
标签
His tag C-Terminus -
额外的序列信息
C-terminal 10xHis-tagged
-
相关产品
-
Related Products
技术指标
Our Abpromise guarantee covers the use of ab235011 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
-
应用
Functional Studies
SDS-PAGE
-
形式
Liquid -
补充说明
Produced in an in vitro E.coli expression system.
Previously labelled as ABCD1.
-
Concentration information loading...
制备和贮存
-
稳定性和存储
Shipped at 4°C. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 7.2
Constituents: Tris buffer, 50% Glycerol (glycerin, glycerine)This product is an active protein and may elicit a biological response in vivo, handle with caution.
常规信息
-
别名
- ABC42
- Abcd1
- ABCD1_HUMAN
see all -
功能
Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity. -
疾病相关
Defects in ABCD1 are the cause of adrenoleukodystrophy X-linked (X-ALD) [MIM:300100]. X-ALD is a peroxisomal metabolic disorder characterized by progressive multifocal demyelination of the central nervous system and by peripheral adrenal insufficiency (Addison disease). It results in mental deterioration, corticospinal tract dysfunction, and cortical blindness. Different clinical manifestations exist like: cerebral childhood ALD (CALD), adult cerebral ALD (ACALD), adrenomyeloneuropathy (AMN) and 'Addison disease only' (ADO) phenotype.
Note=The promoter region of ABCD1 is deleted in the chromosome Xq28 deletion syndrome which involves ABCD1 and the neighboring gene BCAP31. -
序列相似性
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. -
细胞定位
Peroxisome membrane. - Information by UniProt
图片
实验方案
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
数据表及文件
-
SDS download
-
Datasheet download
文献 (0)
ab235011 尚未被引用在任何文献中。