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Rabbit Anti-HMGCS2/PE Conjugated antibody (bs-5070R-PE)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-5070R-PE
英文名稱 Rabbit Anti-HMGCS2/PE Conjugated antibody
中文名稱 PE標記的三羥基三甲基輔酶A合成酶2抗體
別    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 A synthase; HMCS2_HUMAN; HMG CoA synthase; HMG-CoA synthase; HMGCS 2; HMGCS2; Hydroxymethylglutaryl CoA synthase; Hydroxymethylglutaryl CoA synthase mitochondrial; Hydroxymethylglutaryl-CoA synthase; mitochondrial.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  心血管  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 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 52kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HMGCS2
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 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 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) [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 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.

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