三羟基**基辅酶A合成酶2抗体
规格：1mg/1ml
英文名： HMGCS2
别名： 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2 (mitochondrial); 3 hydroxy 3 methylglutaryl Coenzyme A synthase 2; 3 hydroxy 3 methylglutaryl coenzyme A synthase; 3-hydroxy-3-methylglutaryl coenzyme
分子量： 52kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human HMGCS2
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
细胞定位：细胞浆 线粒体
三羟基**基辅酶A合成酶2抗体产品介绍：background: HMGCS2 is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. Function: This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. Subcellular Location: Mitochondrion. Tissue Specificity: High expression in liver and colon. Low expression in testis, heart, skeletal muscle and kidney. DISEASE: Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency)三羟基**基辅酶A合成酶2抗体 [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 三羟基**基辅酶A合成酶2抗体of urinary organic acids with increase of medium and short chain dicarboxylic acids. Similarity: Belongs to the HMG-CoA synthase family. Database links: UniProtKB/Swiss-Prot: P54868.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
三羟基**基辅酶A合成酶2抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 心血管 细胞生物 **学 信号转导 脂蛋白 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid