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

SUCLG1 Antibody, HRP conjugated

  • 中文名稱:
    SUCLG1兔多克隆抗體, HRP偶聯
  • 貨號:
    CSB-PA022919LB01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) SUCLG1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    SUCLG1
  • 別名:
    FLJ21114 antibody; FLJ43513 antibody; GALPHA antibody; MTDPS9 antibody; SCS alpha antibody; SCS-alpha antibody; SUCA_HUMAN antibody; Succinate CoA ligase alpha subunit antibody; Succinyl CoA ligase [GDP forming] subunit alpha mitochondrial antibody; Succinyl CoA synthetase subunit alpha antibody; Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha; mitochondrial antibody; Succinyl-CoA synthetase subunit alpha antibody; SUCLA1 antibody; SUCLG 1 antibody; SUCLG1 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial protein (210-316AA)
  • 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.

產品評價

靶點詳情

  • 功能:
    Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits.
  • 基因功能參考文獻:
    1. We report two Tunisian patients belonging to a consanguineous family with mitochondrial encephalomyopathy. Mutational analysis of SUCLG1 gene showed the presence of c.41T > C in exon 1 in a homozygous state. This mutation substitutes a conserved methionine residue to a threonine at position 14 (p.M14T) located at the SUCLG1 protein mitochondrial targeting sequence. PMID: 29217198
    2. These abnormal phenotypes are rescued upon ectopic expression of wild-type SUCLG1 in the patient's fibroblasts, thus functionally confirming the pathogenic nature of the SUCLG1 VUS identified in this patient and expanding the phenotypic spectrum for SUCLG1 deficiency PMID: 27484306
    3. Long survival, to age 20 years or older, was reported in 12% of SUCLA2 and in 10% of SUCLG1 patients. PMID: 26475597
    4. First Chinese report of succinyl-CoA ligase deficiency caused by novel SUCLG1 mutations; five novel pathogenic mutations in SUCLG1 were identified PMID: 26028457
    5. 3 novel mutations have been identified in patients with the mitochondrial DNA depletion syndrome (c.1048G>A and c.1049G>T in SUCLA2 and c.531+4A>T in SUCLG1). PMID: 22980518
    6. Our results suggest that SUCLG1 mutations that lead to complete absence of SUCLG1 protein are responsible for a very severe disorder with antenatal manifestations, whereas a SUCLA2-like phenotype is found in patients with residual SUCLG1 protein. PMID: 20693550
    7. This report enlarges the phenotypic spectrum of SUCLG1 mutations and confirms that a characteristic metabolic profile (presence of MMA and C4-DC carnitine in urines) and basal ganglia MRI lesions are the hallmarks of SCS defects. PMID: 20197121
    8. A novel mutation in the SUCLG1 gene was found in two newborns having lethal lactic acidosis, multi-organ failure and congenital malformations including interrupted aortic arch. PMID: 20227526

    顯示更多

    收起更多

  • 相關疾病:
    Mitochondrial DNA depletion syndrome 9 (MTDPS9)
  • 亞細胞定位:
    Mitochondrion.
  • 蛋白家族:
    Succinate/malate CoA ligase alpha subunit family
  • 數據庫鏈接:

    HGNC: 11449

    OMIM: 245400

    KEGG: hsa:8802

    STRING: 9606.ENSP00000377446

    UniGene: Hs.270428



主站蜘蛛池模板: 天天摸夜夜摸夜夜狠狠摸 | 亚洲不卡1卡2卡三卡入口| 国产精品人成电影在线观看| 日日碰狠狠添天天爽五月婷| 少妇裸交aa大片| 亚洲国产成人无码网站大全| 可以看三级的网站| 日本一区二区三区免费视频| 国产老熟女老女人老人| 黑人与中国少妇xxxx视频| 中文字幕人妻无码一夲道| 亚洲欧洲日产国码无码av一| 久久久久人妻精品一区三寸| 国产区又黄又硬高潮的视频| 亚洲精品国产美女久久久99| 99久久er热在这里只有精品99| 国产乱子伦农村xxxx| 国内精品久久久久伊人av| 精品麻豆一区二区三区乱码| 香蕉97超级碰碰碰免费公开| 999精品无码a片在线1级| 国产精品无码无片在线观看| 日本午夜免费福利视频| 手机看片国产av无码| 日本熟妇美熟bbw| 欧美亚洲国产片在线播放| 国産精品久久久久久久| 夜夜爽狠狠天天婷婷五月| 日日摸日日碰夜夜爽免费| 亚洲一区二区三区在线网址| 亚洲大尺度无码专区尤物| 亚洲综合无码久久精品综合| 少妇的丰满3中文字幕| 激情综合婷婷丁香五月情| 无码专区—va亚洲v专区| 黄网在线观看免费网站| 日本强伦姧人妻一区二区| 久久婷婷六月综合色液啪| 国产欧美另类久久久精品不卡| 99国产精品久久久久久久日本竹| а√天堂资源中文在线官网|