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        HMGCS2 Rabbit pAb (bs-5070R)  
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        產品編號 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
        克 隆 號
        交叉反應 Mouse,Rat (predicted: Human,Rabbit,Pig,Cow,Dog,Horse)
        產品應用 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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