在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. PCCA Antibody, HRP conjugated

PCCA Antibody, HRP conjugated

  • 中文名稱：
    PCCA兔多克隆抗體, HRP偶聯(lián)
  • 貨號：
    CSB-PA017522LB01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) PCCA Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    mitochondrial antibody; Pcca antibody; pccA COMPLEMENTATION GROUP antibody; PCCA_HUMAN antibody; PCCase subunit alpha antibody; Propanoyl CoA:carbon dioxide ligase subunit alpha antibody; Propanoyl-CoA:carbon dioxide ligase subunit alpha antibody; Propionyl CoA carboxylase alpha chain antibody; Propionyl CoA carboxylase alpha chain mitochondrial antibody; Propionyl CoA carboxylase alpha polypeptide antibody; Propionyl coenzyme A carboxylase alpha polypeptide antibody; Propionyl-CoA carboxylase alpha chain antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Propionyl-CoA carboxylase alpha chain, mitochondrial protein (585-720AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    HRP
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應用范圍：
    ELISA
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

  • 功能：
    This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA.
  • 基因功能參考文獻：
    1. The majority of patients had mutations in the PCCA gene (18/25). A total of 26 mutations were noted: 20 in the PCCA gene and 6 in PCCB gene. Seventeen mutations were novel (14 in PCCA and 3 in PCCB). The SNP c.937C>T (p.Arg313Ter), was noted in 9/36 (25%) alleles in the PCCA gene PMID: 27227689
    2. Ten propionic acidemia mutations were confirmed, including 8 affecting the PCCA gene and 2 affecting the PCCB gene PMID: 25636094
    3. Two PCCA mutations, c.229C-->T (p.R77W) and c.1262A-->C (p.Q421P), were identified in a PCCA-deficient patient. PMID: 24863100
    4. Mutation analysis of the PCCA gene identified homozygous c.1284+1G>A in patient 1, c.230G>A (p.R77Q) and c.1855C>T (p.R619X) in patient 2, homozygous c.2125T>C (p.S709P) in patient 3, and only one mutant allele, c.231+1G>T in patient 4. PMID: 24464666
    5. Thsi study presented that Propionic acidemia(PCCA mutation) associated with visual hallucinations PMID: 22156789
    6. propionyl CoA carboxylase alpha polypeptide intronic variations causing aberrantly spliced messenger RNA is associated with propionic and methylmalonic acidemia. PMID: 17966092
    7. cryo-electron microscopy (cryo-EM) reconstruction at 15-A resolution PMID: 20725044
    8. pathogenicity of 11 mutations by expression studies and correlation of genotype-phenotype in PCCA-deficient propionic acidemia patients. PMID: 12385775
    9. Data reported 9 novel PCCA gene mutations and represents an extensive update of the mutational study of propionic acidemia providing important information about the worldwide distribution of PA mutations. PMID: 12559849
    10. analysis of propionyl-CoA carboxylase containing pathogenic mutations in the beta subunit (R165W, E168K, and R410W) and one PCCB polymorphism (A497V) and their structural and functional effects PMID: 15890657
    11. analysis of PCCA and PCCB mutations in propionic acidemia PMID: 17051315
    12. This work describes for the first time the high frequency of large genomic deletions in the PCCA gene, which could be due to the characteristics of the PCCA gene structure and its abundance in intronic repetitive elements. PMID: 19157943
    13. Activities of propionyl-CoA carboxylase were not significantly different in pancreatic islets of patients with type 2 diabetes from those of the control. PMID: 19296078

    顯示更多

    收起更多

  • 相關疾病：
    Propionic acidemia type I (PA-1)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 8653

    OMIM: 232000

    KEGG: hsa:5095

    STRING: 9606.ENSP00000365462

    UniGene: Hs.733632



主站蜘蛛池模板： 欧美一性一乱一交一视频| 国产精品美女久久久久久久久| 扒开双腿吃奶呻吟做受视频 | 国产亚洲精品久久yy50| 国产精品十八禁在线观看| 久久综合九色综合欧美狠狠| 无码中字出轨中文人妻中文中| 在线 亚洲 国产 欧美| 又爽又黄又无遮挡的激情视频| 少妇仑乱a毛片| 97精品一区二区视频在线观看| 亚洲va综合va国产产va中文| 欧美人与禽z0zo牲伦交| 久久久久久久久久久久久9999| 亚洲中文字幕无码一区在线 | 丁香五月缴情在线| 四虎成人国产精品永久在线| 国产偷窥熟妇高潮呻吟| 无码人妻精品一区二区蜜桃网站| 精品少妇人妻av久久久| 国产亚洲日韩在线a不卡| 人妻少妇中文字幕乱码| 亚洲国色天香卡2卡3卡4| 亚洲444kkkk在线观看| 亚洲精品中国国产嫩草影院美女 | 内射干少妇亚洲69xxx| 日本亚洲欧美高清专区vr专区| 久久久久人妻精品区一三寸| 亚洲 日本 欧美 中文幕| 亚洲日韩一页精品发布| 秋霞av在线露丝片av无码| 真实国产乱子伦对白在线播放| 国产精品水嫩水嫩| 精品熟女少妇a∨免费久久| 亚洲精品四区麻豆文化传媒| 少妇仑乱a毛片| 免费大黄网站| 亚洲中文有码字幕日本| 亚洲无人区一区二区三区| 国产网红主播无码精品| 国产成人精品三级在线影院|