重组人HIBCH蛋白(ab124585)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE, MS
描述
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产品名称
重组人HIBCH蛋白 -
纯度
> 90 % SDS-PAGE.
ab124585 is purified using conventional chromatography techniques. -
表达系统
Escherichia coli -
Accession
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蛋白长度
Full length protein -
无动物成分
No -
性质
Recombinant -
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种属
Human -
序列
MGSSHHHHHH SSGLVPRGSH MGSHMDAAEE VLLEKKGCTG VITLNRPKFL NALTLNMIRQ IYPQLKKWEQ DPETFLIIIK GAGGKAFCAG GDIRVISEAE KAKQKIAPVF FREEYMLNNA VGSCQKPYVA LIHGITMGGG VGLSVHGQFR VATEKCLFAM PETAIGLFPD VGGGYFLPRL QGKLGYFLAL TGFRLKGRDV YRAGIATHFV DSEKLAMLEE DLLALKSPSK ENIASVLENY HTESKIDRDK SFILEEHMDK INSCFSANTV EEIIENLQQD GSSFALEQLK VINKMSPTSL KITLRQLMEG SSKTLQEVLT MEYRLSQACM RGHDFHEGVR AVLIDKDQSP KWKPADLKEV TEEDLNNHFK SLGSSDLKF -
预测分子量
42 kDa including tags -
氨基酸
33 to 386 -
标签
His tag N-Terminus
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相关产品
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Related Products
技术指标
Our Abpromise guarantee covers the use of ab124585 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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应用
SDS-PAGE
Mass Spectrometry
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质谱法
MALDI-TOF -
形式
Liquid -
Concentration information loading...
制备和贮存
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稳定性和存储
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
pH: 8.00
Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol (glycerin, glycerine), 1.17% Sodium chloride
常规信息
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别名
- 3 hydroxyisobutyryl Coenzyme A hydrolase
- 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial
- 3-hydroxyisobutyryl-CoA hydrolase
see all -
功能
Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA. -
组织特异性
Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung. -
通路
Amino-acid degradation; L-valine degradation. -
疾病相关
Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:250620]; also known as deficiency of beta-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. The enzyme defect results in accumulation of methacrylyl-CoA, a highly reactive compound, which readily undergoes addition reactions with free sulfhydryl groups. Affected individuals showed delayed development of motor skills, hypotonia, initial poor feeding, and a deterioration in neurological function during first stages of life. -
序列相似性
Belongs to the enoyl-CoA hydratase/isomerase family. -
细胞定位
Mitochondrion. - Information by UniProt
图片
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SDS-PAGE - Recombinant Human HIBCH protein (ab124585)15% SDS-PAGE showing ab124585 at approximately 42.1kDa (3µg).
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
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SDS download
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Datasheet download
文献 (0)
ab124585 尚未被引用在任何文献中。