三羟基**基辅酶A裂解酶抗体
规格：1mg/1ml
英文名： HMGCL
别名： 3 hydroxy 3 methylglutaryl CoA lyase; 3 hydroxy 3 methylglutaryl Coenzyme A lyase; 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase (hydroxymethylglutaricaciduria); 3 hydroxymethyl 3 methylglutaryl C
分子量： 32kDa
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 HMGCL
交叉反应：Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit,
细胞定位：细胞浆 线粒体
三羟基**基辅酶A裂解酶抗体产品介绍：background: Hydroxymethylglutaryl-CoA lyase (HMGCL) is found in fibroblasts, liver and lymphoblasts. It has a role in ketogenesis and leucine catabolism. Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (hydroxymethylglutaricaciduria), an autosomal recessive disease which can lead to hypoglycemia and coma. Function: Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Subunit: Homodimer; disulfide-linked. Can also form homotetramers. Subcellular Location: Mitochondrion matrix. Tissue Specificity: Fibroblasts, liver and lymphoblasts. DISEASE: Defects三羟基**基辅酶A裂解酶抗体 in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic 三羟基**基辅酶A裂解酶抗体hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases. Similarity: Belongs to the HMG-CoA lyase family. Database links: UniProtKB/Swiss-Prot: P35914.2 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裂解酶抗体产品应用：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 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