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Rabbit Anti-HMGCL/Gold Conjugated antibody (bs-5067R-Gold)
訂購熱線:400-901-9800
訂購郵箱:sales@m.p2b3.cn
訂購QQ:  400-901-9800
技術支持:techsupport@m.p2b3.cn
說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-5067R-Gold
英文名稱1 Rabbit Anti-HMGCL/Gold Conjugated antibody
中文名稱 膠體金標記的三羥基三甲基輔酶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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 腫瘤  細胞生物  免疫學  信號轉(zhuǎn)導  脂蛋白  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit, )
產(chǎn)品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 32kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HMGCL
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產(chǎn)品介紹 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 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.

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.
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