三羥基三甲基輔酶A裂解酶抗體

產品貨號：

BN41038R
中文名稱：

三羥基三甲基輔酶A裂解酶抗體
英文名稱：

Rabbit anti-HMGCL Polyclonal antibody
品牌：

Biorigin

貨號

產品規格

售價

備注
BN41038R-100ul

100ul

￥2360.00

交叉反應：Mouse(predicted:Human,Rat,Chicken,Dog,Horse,Rabbit) 推薦應用：WB,IHC-P,IHC-F,IF,ELISA
BN41038R-200ul

200ul

￥3490.00

交叉反應：Mouse(predicted:Human,Rat,Chicken,Dog,Horse,Rabbit) 推薦應用：WB,IHC-P,IHC-F,IF,ELISA

產品描述

英文名稱	HMGCL
中文名稱	三羥基三甲基輔酶A裂解酶抗體
別名	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 Coenzyme A lyase; 3-hydroxy-3-methylglutarate-CoA lyase; HL; HMG CoA lyase; HMG CoA Lyase Deficiency; HMG-CoA lyase; HMGCL; HMGCL_HUMAN; Hydroxymethylglutaricaciduria; Hydroxymethylglutaryl CoA lyase; Hydroxymethylglutaryl CoA lyase mitochondrial; Hydroxymethylglutaryl-CoA lyase; mitochondrial; MS725; OTTHUMP00000044830.
研究領域	腫瘤細胞生物免疫學信號轉導脂蛋白線粒體
抗體來源	Rabbit
克隆類型	Polyclonal
交叉反應	Mouse, (predicted: Human, Rat, Chicken, Dog, Horse, Rabbit, )
產品應用	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
分子量	32kDa
細胞定位	細胞漿線粒體
性狀	Liquid
濃度	1mg/ml
免疫原	KLH conjugated synthetic peptide derived from human HMGCL:41-140/325
亞型	IgG
純化方法	affinity purified by Protein A
儲存液	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMed	PubMed
產品介紹	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 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 hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases. Similarity: Belongs to the HMG-CoA lyase family. SWISS: P35914 Gene ID: 3155 Database links: Entrez Gene: 3155 Human Entrez Gene: 15356 Mouse Entrez Gene: 79238 Rat Omim: 246450 Human Omim: 613898 Human SwissProt: P35914 Human SwissProt: P38060 Mouse SwissProt: P97519 Rat Unigene: 533444 Human Unigene: 482102 Mouse Unigene: 12297 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.