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. 首頁
  2. 蛋白
  3. 重組蛋白
  4. Recombinant Human Paraplegin (SPG7), partial

Recombinant Human Paraplegin (SPG7), partial

  • 中文名稱：
    人SPG7重組蛋白
  • 貨號：
    CSB-YP892174HU1
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人SPG7重組蛋白
  • 貨號：
    CSB-EP892174HU1
  • 規格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    人SPG7重組蛋白
  • 貨號：
    CSB-EP892174HU1-B
  • 規格：
  • 來源：
    E.coli
  • 共軛：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名稱：
    人SPG7重組蛋白
  • 貨號：
    CSB-BP892174HU1
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人SPG7重組蛋白
  • 貨號：
    CSB-MP892174HU1
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    SPG7
  • Uniprot No.：
  • 別名：
    SPG7; CAR; CMAR; PGN; Paraplegin; Cell matrix adhesion regulator; Spastic paraplegia 7 protein
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Partial
  • 蛋白標簽：
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

產品評價

靶點詳情

  • 功能：
    ATP-dependent zinc metalloprotease. Plays a role in the formation and regulation of the mitochondrial permeability transition pore (mPTP) and its proteolytic activity is dispensable for this function.
  • 基因功能參考文獻：
    1. Compound heterozygous variants in SPG7 identified in 22 French Canadian patients with spastic ataxia. PMID: 26626314
    2. CACNA1A and SPG7 are major ataxia genes. PMID: 28444220
    3. The results of this study showed that the most frequently detected variant in this cohort was the SPG7 p.Leu78. PMID: 27084228
    4. A Norwegian founder mutation p.H701P is a major cause of SPG7 in Norway. PMID: 26756429
    5. a novel homozygous frameshift deletion in the SPG7 gene was identifies as the genetic cause of hereditary spastic paraplegia in a Greek family. PMID: 26260707
    6. this case shows that the spectrum of pathologies in SPG7 can include neuron loss of the dentate nucleus and the inferior olivary nucleus as well as neuritic pathology. PMID: 26506339
    7. Data indicates that SPG7 is essential for the mitochondrial permeability transition pore (PTP) complex formation, interacts with CypD and VDAC and determines C terminus of SPG7 and CsA-binding region of CypD as necessary for PTP formation. PMID: 26387735
    8. In unexplained ataxia, there was a significant number of patients with SPG7 mutations. PMID: 25681447
    9. The SPG7 Q866 variant is efficiently processed independent of phosphorylation of AFG3L2 at Y179, which inhibits processing of SPG7. PMID: 24767997
    10. Using an unbiased exome sequencing approach we identified pathogenic compound heterozygous SPG7 mutations in patients with PEO and multiple mitochondrial DNA deletions in skeletal muscle PMID: 24727571
    11. A Japanese patient is reported with an SPG7 mutation for a slowly progressive form of autosomal recessive cerebellar ataxia and spastic paraplegia. PMID: 23857099
    12. This study showed that the p.Ala510Val mutation is prevalent amongst severe hereditary spastic paraparesis patients of UK. PMID: 23269439
    13. Data suggest a pathogenic role for this SPG7 p.A510V variant. PMID: 22571692
    14. SPG7 mutations correlate with spastic paraplegia phenotypes. PMID: 22964162
    15. SPG7 mutations are a frequent cause of middle-aged onset of spastic gait when strict inclusion criteria are applied and should, therefore, be tested in autosomal recessive or sporadic hereditary spastic paraplegia. PMID: 23065789
    16. Studies indicate that both mouse and human SPG7 ESTs containing alternative first exons. PMID: 22563492
    17. The novel mutation is the first splice site mutation found in the SPG7 gene. It removes part of the AAA domain of paraplegin protein, probably leading to a loss-of-function of the paraplegin-AFG3L2 complex in the mitochondrial inner membrane. PMID: 20108356
    18. structural analysis of the ATPase domain of the human AAA+ protein paraplegin/SPG7 PMID: 19841671
    19. Adenoassociated virus-mediated intramuscular delivery of paraplegin halted the progression of neuropathological changes and rescued mitochondrial morphology in the peripheral nerves of paraplegin-deficient mice. PMID: 16357941
    20. Cerebellar signs or cerebellar atrophy on brain imaging were the most frequent additional features in patients with SPG7 hereditary spastic paraplegia. PMID: 16534102
    21. The new SPG7 gene mutation leads to a novel complicated autosomal recessive hereditary spastic paraparesis phenotype that widens the spectrum of different brain systems that are optionally affected in hereditary spastic paraplegia. PMID: 17646629
    22. identification of six novel point mutations and one large intragenic deletion in hereditary spastic paraplegia PMID: 18200586
    23. This study identified a novel paraplegin mutation, c.1047insC, in a non-consanguineous Norwegian family with ARHSP. PMID: 18563470
    24. Results suggest that paraplegin mutations are a frequent cause of sporadic spastic paraparesis. PMID: 18799786
    25. An intersubunit signaling network coordinates ATP hydrolysis by m-AAA protease paraplegin. PMID: 19748354

    顯示更多

    收起更多

  • 相關疾病：
    Spastic paraplegia 7, autosomal recessive (SPG7)
  • 亞細胞定位：
    Mitochondrion inner membrane; Multi-pass membrane protein.
  • 蛋白家族：
    AAA ATPase family; Peptidase M41 family
  • 組織特異性：
    Ubiquitous.
  • 數據庫鏈接：

    HGNC: 11237

    OMIM: 602783

    KEGG: hsa:6687

    STRING: 9606.ENSP00000268704

    UniGene: Hs.185597



主站蜘蛛池模板： 欧美黑人疯狂性受xxxxx喷水| 日本三级香港三级人妇三| 亚洲精品综合在线影院| 国产成人无码精品久久涩吧| 宝贝腿开大点我添添公视频免费| 可以直接免费观看的av网站| 性生交大片免费看l| 在教室伦流澡到高潮hgl动漫| 96亚洲精华国产精华精华液| 日本乱偷人妻中文字幕在线| 国产高清av在线播放| 亚洲人成77777在线播放网站| 欧美特大特白屁股ass| 在线看片人成视频免费无遮挡| 免费男人下部进女人下部视频| 欧美激情精品成人一区| 久久精品第九区免费观看| 熟女内射v888av| 国产成人综合久久精品推下载| 综合色区国产亚洲另类| 欧美成人国产精品高潮| 天天天欲色欲色www免费| 国产美女自卫慰水免费视频| 2021无码天堂在线| 天堂www中文在线资源| 老妇高潮潮喷到猛进猛出| 成人做爰高潮片免费视频| 在线观看成人无码中文av天堂 | 99精品全国免费观看视频| 午夜人妻久久久久久久久| 欧美老熟妇又粗又大| 9999国产精品欧美久久久久久| 免费看又色又爽又黄的国产软件| 麻豆亚洲一区| av无码国产精品麻豆| 亚洲欧美中文日韩v在线97| 亚洲午夜成人片| 真实国产乱子伦对白视频不卡| 亚洲色欲av无码成人专区| 中文字幕人妻色偷偷久久| 国产精品爽爽久久久久久竹菊|