重组人HMGCS2蛋白(ab114900)
Key features and details
- Expression system: Wheat germ
- Suitable for: WB, SDS-PAGE, ELISA
描述
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产品名称
重组人HMGCS2蛋白 -
表达系统
Wheat germ -
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
MQRLLTPVKRILQLTRAVQETSLTPARLLPVAHQRFSTASAVPLAKTDTW PKDVGILALEVYFPAQYVDQTDLEKYNNVEAGKYTVGLGQTRMGFCSVQE DINSLCLTVVQRLMERIQLPWDSVGRLEVGTETIIDKSKAVKTVLMELFQ DSGNTDIEGIDTTNACYGGTASLFNAANWMESSSWDGRYAMVVCGDIAVY PSGNARPTGGAGAVAMLIGPKAPLALERGLRGTHMENVYDFYKPNLASEY PIVDGKLSIQCYLRALDRCYTSYRKKIQNQWKQAGSDRPFTLDDLQYMIF HTPFCKMVQKSLARLMFNDFLSASSDTQTSLYKGLEAFGGLKLEDTYTNK DLDKALLKASQDMFDKKTKASLYLSTHNGNMYTSSLYGCLASLLSHHSAQ ELAGSRIGAFSYGSGLAASFFSFRVSQDAAPGSPLDKLVSSTSDLPKRLA SRKCVSPEEFTEIMNQREQFYHKVNFSPPGDTNSLFPGTWYLERVDEQHR RKYARRPV -
预测分子量
82 kDa including tags -
氨基酸
1 to 508
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技术指标
Our Abpromise guarantee covers the use of ab114900 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
Western blot
SDS-PAGE
ELISA
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形式
Liquid -
Concentration information loading...
制备和贮存
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稳定性和存储
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00
Constituents: 0.3% Glutathione, 0.79% Tris HCl
常规信息
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别名
- 3 hydroxy 3 methylglutaryl coenzyme A synthase
- 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2
- 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2 (mitochondrial)
see all -
功能
This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. -
组织特异性
High expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney. -
通路
Metabolic intermediate biosynthesis; (R)-mevalonate biosynthesis; (R)-mevalonate from acetyl-CoA: step 2/3. -
疾病相关
Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency) [MIM:605911]; also known as deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase 2. Affected individuals present with severe hypoketotic hypoglycemia, mild hepatomegaly, or fatty liver, and a nondiagnostic pattern of urinary organic acids with increase of medium and short chain dicarboxylic acids. -
序列相似性
Belongs to the HMG-CoA synthase family. -
细胞定位
Mitochondrion. - Information by UniProt
图片
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SDS-PAGE - Recombinant Human HMGCS2 protein (ab114900)12.5% SDS-PAGE showing ab114900 at approximately 81.95kDa.
Stained with Coomassie Blue.
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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Datasheet download
文献 (1)
ab114900 被引用在 1 文献中.
- Bai M et al. LONP1 targets HMGCS2 to protect mitochondrial function and attenuate chronic kidney disease. EMBO Mol Med 15:e16581 (2023). PubMed: 36629048
