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  4. Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial(MCCC1) ELISA kit

Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial(MCCC1) ELISA kit

  • 中文名稱：
    人甲基丁烯酰輔酶A羧化酶亞基α，線粒體(MCCC1)酶聯免疫試劑盒
  • 貨號：
    CSB-EL013572HU
  • 規格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 其他：

產品詳情

  • 產品描述：
    人甲基丁烯酰輔酶A羧化酶亞基α，線粒體(MCCC1)酶聯免疫試劑盒（CSB-EL013572HU）為雙抗夾心法ELISA試劑盒，定量檢測組織勻漿、細胞裂解物樣本中的MCCC1含量。MCCC1作為研究靶點，它在線粒體代謝中扮演關鍵角色。背景上，其功能與多種代謝過程相關。研究機制方面，聚焦于它在代謝通路中的具體作用，探究其如何參與物質轉化和能量代謝，以及異常時與疾病發生發展的關聯。試劑盒檢測范圍為0.78 ng/ml-50ng/ml，該產品適用于基礎醫學研究中MCCC1蛋白表達的定量分析，例如代謝性疾病模型構建、線粒體功能評估、細胞能量代謝調控機制研究等領域，為探索遺傳代謝缺陷、營養代謝異常等課題提供可靠的實驗工具。本品僅用于科研，不用于臨床診斷，產品具體參數及操作步驟詳見產品說明書。
  • 別名：
    MCCC1 ELISA Kit; MCCAMethylcrotonoyl-CoA carboxylase subunit alpha ELISA Kit; mitochondrial ELISA Kit; MCCase subunit alpha ELISA Kit; EC 6.4.1.4 ELISA Kit; 3-methylcrotonyl-CoA carboxylase 1 ELISA Kit; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit ELISA Kit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha ELISA Kit
  • 縮寫：
    MCCC1
  • Uniprot No.：
  • 種屬：
    Homo sapiens (Human)
  • 樣本類型：
    tissue homogenates, cell lysates
  • 檢測范圍：
    0.78 ng/ml-50ng/ml
  • 靈敏度：
    0.19 ng/ml
  • 反應時間：
    1-5h
  • 樣本體積：
    50-100ul
  • 檢測波長：
    450 nm
  • 研究領域：
    Metabolism
  • 測定原理：
    quantitative
  • 測定方法：
    Sandwich
  • 線性度：
  • 數據處理：
  • 貨期：
    3-5 working days

產品評價

靶點詳情

  • 功能：
    Biotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
  • 基因功能參考文獻：
    1. MCCC1 plays an essential role in virus-triggered, MAVS-mediated activation of NF-kappaB signaling. PMID: 27629939
    2. This study reports data mainly obtained from the Portuguese newborn screening program collected over a ten-year period. Analysis of the MCCC1 and MCCC2 genes yielded 26 previously unreported mutations and a variant of clinically unknown significance. PMID: 27601257
    3. Our study provides strong support for the susceptibility role of RAB7L1/NUCKS1 rs823118 and MCCC1 rs12637471 in sporadic Parkinson's disease in a Han Chinese population PMID: 26914237
    4. Novel mutations in MCCC1 gene were identified in Chinese population. The expression profiles of two splice mutations (c.639+2T>A and c.639+5G>T) were also characterized. PMID: 25382614
    5. Four new point mutations were detected in the MCCC1 gene in patients with maternal 3-methylcrotonyl coenzyme deficiency. PMID: 24078573
    6. This study demonistrated that Mainland China demonstrates that MCCC1/LAMP3 (rs11711441) is associated with a lower risk of Parkinson's disease. PMID: 23496138
    7. 3-methylcrotonyl-CoA carboxylase inhibition has a role in increased excretion of 3-hydroxyisovaleric acid in valproate-treated patients PMID: 22189597
    8. Mutation in 3-methylcrotonyl CoA carboxylase 1 gene is associated with 3-methylcrotonyl-CoA carboxylase deficiency. PMID: 22264772
    9. study reports eight different mutant alleles of MCCC1 or MCCC2 including six novel mutations in Korean patients with 3-methylcrotonyl-CoA carboxylase (MCC) deficiency PMID: 22150417
    10. identified two novel MCCA and four novel MCCB mutant alleles from five MCC-deficient patients PMID: 21071250
    11. factors other than the genotype at the MCCA and MCCB loci have a major influence on the phenotype of MCC deficiency PMID: 16010683
    12. The amino-termini containing 39 (MCCalpha) or 20 amino acids (MCCbeta) were both necessary and sufficient for targeting. Structural requirements for mitochondrial import were defined by site-directed mutagenesis. PMID: 16023992
    13. Molecular analyses revealed novel mutations in one of the causative genes, MCCA or MCCB, in all five of the MCC deficiency patients PMID: 17968484
    14. A family with 3-methylcrotonyl-CoA carboxylase deficiency with different clinical features is described. PMID: 19339287
    15. analysis of a novel mechanism causing 3-methylcrotonyl-CoA carboxylase deficiency PMID: 19706617

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  • 相關疾病：
    3-methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 數據庫鏈接：

    HGNC: 6936

    OMIM: 210200

    KEGG: hsa:56922

    STRING: 9606.ENSP00000265594

    UniGene: Hs.47649



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