<address id="cansv"></address>
<s id="cansv"><sub id="cansv"></sub></s>
    <s id="cansv"></s>
    <span id="cansv"><input id="cansv"></input></span>
      <label id="cansv"></label>
      <s id="cansv"></s>

      <strike id="cansv"></strike>

      1. <s id="cansv"><sub id="cansv"><rt id="cansv"></rt></sub></s>
      2. <label id="cansv"></label>
      3. 掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
          
        客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
        產品中心-北京博奧森生物技術有限公司
        首頁 > 產品中心 > 一抗 > 產品信息
        IVD Rabbit pAb (bs-17189R)  
        訂購熱線:400-901-9800
        訂購郵箱:sales@bioss.com.cn
        訂購QQ:  400-901-9800
        技術支持:techsupport@bioss.com.cn
        50ul/1180.00元
        100ul/1980.00元
        200ul/2800.00元
        大包裝/詢價

        產品編號 bs-17189R
        英文名稱 IVD Rabbit pAb
        中文名稱 異戊酰輔酶A脫氫酶抗體
        別    名 ACAD2; FLJ12715; FLJ34849; Isovaleryl CoA dehydrogenase; Isovaleryl CoA dehydrogenase, mitochondrial; Isovaleryl Coenzyme A dehydrogenase; Isovaleryl-CoA dehydrogenase; IVD; IVD_HUMAN; mitochondrial.  
        研究領域 腫瘤  細胞生物  神經生物學  信號轉導  新陳代謝  干擾素  
        抗體來源 Rabbit
        克隆類型 Polyclonal
        克 隆 號
        交叉反應 Mouse (predicted: Human,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse)
        產品應用 WB=1:500-2000
        not yet tested in other applications.
        optimal dilutions/concentrations should be determined by the end user.
        理論分子量 43 kDa
        檢測分子量
        細胞定位 細胞漿 
        性    狀 Liquid
        濃    度 1mg/ml
        免 疫 原 KLH conjugated synthetic peptide derived from human IVD: 201-300/423 
        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 Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009]

        Function:
        Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 1/3.

        Subcellular Location:
        Mitochondrion matrix.

        DISEASE:
        Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:243500]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.

        Similarity:
        Belongs to the acyl-CoA dehydrogenase family.

        SWISS:
        P26440

        Gene ID:
        3712

        Database links:

        Entrez Gene: 3712 Human

        Entrez Gene: 510440 Cow

        Entrez Gene: 56357 Mouse

        Entrez Gene: 100156047 Pig

        Entrez Gene: 24513 Rat

        Omim: 607036 Human

        SwissProt: Q3SZI8 Cow

        SwissProt: P26440 Human

        SwissProt: Q9JHI5 Mouse

        SwissProt: P12007 Rat

        Unigene: 513646 Human

        Unigene: 6635 Mouse

        Unigene: 147 Rat



        產品圖片
        25 ug total protein per lane of various lysates (see on figure) probed with IVD polyclonal antibody, unconjugated (bs-17189R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
        版權所有 2004-2026 m.radiasunchina.com 北京博奧森生物技術有限公司
        通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
        通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
        京ICP備05066980號-1         京公網安備110107000727號
        国产剧情演绎系列丝袜高跟|一级毛片av性爱黄色网站|欧美三级午夜理伦三级|国产av巨作情欲放纵|亚洲 欧美 中文 日韩aⅴ
        <address id="cansv"></address>
        <s id="cansv"><sub id="cansv"></sub></s>
        <s id="cansv"></s>
        <span id="cansv"><input id="cansv"></input></span>
          <label id="cansv"></label>
          <s id="cansv"></s>

          <strike id="cansv"></strike>

          1. <s id="cansv"><sub id="cansv"><rt id="cansv"></rt></sub></s>
          2. <label id="cansv"></label>