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三羥基三甲基輔酶A裂解酶抗體
  • 產(chǎn)品貨號(hào)：
    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

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  • BN41038R-100ul

    100ul

    ￥2360.00

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

  • BN41038R-200ul

    200ul

    ￥3490.00

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

產(chǎn)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(lǐng)域腫瘤  細(xì)胞生物  免疫學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  脂蛋白  線粒體  
抗體來(lái)源Rabbit
克隆類型Polyclonal
交叉反應(yīng)Mouse,  (predicted: Human, Rat, Chicken, Dog, Horse, Rabbit, )
產(chǎn)品應(yīng)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(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量32kDa
細(xì)胞定位細(xì)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(chǔ) 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.
PubMedPubMed
產(chǎn)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.