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HMGCS2 Rabbit pAb (bs-5070R)  
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50ul/1180.00元
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200ul/2800.00元
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產(chǎn)品編號 bs-5070R
英文名稱 HMGCS2 Rabbit pAb
中文名稱 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.  
Specific References  (1)     |     bs-5070R has been referenced in 1 publications.
[IF=5.072] Ni H et al.ErbB4 acts as a suppressor in colitis and its associated carcinoma by negatively regulating cholesterol metabolism.(2018)Carcinogenesis.Nov 17.  IP&WB ;  Human.  
研究領(lǐng)域 腫瘤  心血管  細胞生物  免疫學(xué)  信號轉(zhuǎn)導(dǎo)  脂蛋白  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應(yīng) Mouse,Rat (predicted: Human,Rabbit,Pig,Cow,Dog,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 52 kDa
檢測分子量
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 HMGCS2: 101-200/508 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
P54868

Gene ID:
3158

Database links:
UniProtKB/Swiss-Prot: P54868.1

產(chǎn)品圖片
Tissue/cell: rat colitis tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-HMGCS2 Polyclonal Antibody, Unconjugated(bs-5070R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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