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

Recombinant Human Collagen alpha-2 (VI) chain (COL6A2), partial

  • 中文名稱:
    Recombinant Human Collagen alpha-2 (VI) chain(COL6A2),partial
  • 貨號:
    CSB-EP005752HU
  • 規格:
    ¥1836
  • 圖片:
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他:

產品詳情

  • 純度:
    Greater than 85% as determined by SDS-PAGE.
  • 基因名:
  • Uniprot No.:
  • 別名:
    CO6A2_HUMAN; COL6A2; Collagen alpha 2(VI) chain; Collagen alpha-2(VI) chain; collagen type VI alpha 2; Collagen VI alpha 2 polypeptide; human mRNA for collagen VI alpha 2 C terminal globular domain; PP3610
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    Partial
  • 來源:
    E.coli
  • 分子量:
    13.4 kDa
  • 表達區域:
    941-1016aa
  • 氨基酸序列
    ELSFVFLTDGVTGNDSLHESAHSMRKQNVVPTVLALGSDVDMDVLTTLSLGDRAAVFHEKDYDSLAQPGFFDRFIR
    Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
    If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
  • 蛋白標簽:
    N-terminal 10xHis-tagged and C-terminal Myc-tagged
  • 產品提供形式:
    Liquid or 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.
  • 緩沖液:
    Tris-based buffer,50% glycerol
  • 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:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. 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 & COA:
    Please contact us to get it.

產品評價

靶點詳情

  • 功能:
    Collagen VI acts as a cell-binding protein.
  • 基因功能參考文獻:
    1. binding of collagen VI to NG2 is essential for the direction of tendon fibroblasts migration in vitro. PMID: 26944560
    2. Genetic study showed a missense mutation in COL6A2 (c.820 G>A, p.Gly268Ser) that causes a glycine substitution in the Gly-X-Y collagenous motif, at the beginning of the collagenous triple helical domain. The c.820 G>A mutation segregated in all the affected patients. PMID: 27563703
    3. Mutations in COL6A2 gene are associated with aberrant mitochondria in Bethlem myopathy. PMID: 25533456
    4. In UCMD, 8 mutations were identified in COL6A2 in Chinese patients. PMID: 24801232
    5. COL6A2 is overexpressed in Down syndrome-affected umbilical cords at early and term gestational ages. PMID: 23452080
    6. Homozygous COL6A2 mutation, p.Asp215Asn, was identified in both affected siblings. We conclude that the COL6A2 p.Asp215Asn mutation is likely to be responsible for PME (Progressive Myoclonus Epilepsy) in this family. PMID: 23138527
    7. A deletion within intron 1A of the COL6A2 gene, occurring in compound heterozygosity with a small deletion in exon 28, was identified in a BM patient. PMID: 20302629
    8. the C2A splice variant has a role in recessive COL6A2 C-globular missense mutations in Ullrich congenital muscular dystrophy PMID: 20106987
    9. The alpha2(VI) chain modulates matrix-metalloproteinase (MMP) availability by sequestering proMMPs in the extracellular matrix, blocking proteolytic activity. PMID: 19698785
    10. the C-terminal globular domain of COL6A2 is not essential for triple-helix formation but is critical for microfibrillar assembly in Ullrich congenital muscular dystrophy PMID: 12218063
    11. A case of Ullrich disease is associated with complete deficiency of collagen VI and compound heterozygous mutations in the collagen VI alpha 2 gene with absence of microfibrils on electron microscopy. PMID: 12297580
    12. Bethlem myopathy is an autosomal dominantly inherited myopathy with contractures, caused by mutations in COL6A1 gene, COL6A2 gene or COL6A3 gene. PMID: 12374585
    13. In Ullrich syndrome, a heterozygous G-to-A substitution at position +5 in intron 23 & the corresponding heterozygous 6-bp deletion in exon 26 which deleted 1 of the 2 tandem repeats of the sequence CATCGG in nt 2268-2273 & 2274-2279 in COL6A2 ORF. PMID: 14981181
    14. diminished COL6A2 mRNA expression found to be primary pathogenic mechanism in UCMD patient PMID: 16075202
    15. This study demonstrates a homogenoeous overexpression of the genes encoding for alpha1 and alpha2 chains of collagen type VI in nuchal skin of human trisomy 21 fetuses. PMID: 17602442
    16. Results describe the characteristic features of myosclerosis myopathy with a homozygous collagen type 6A2 mutation responsible for a peculiar pattern of collagen VI defects. PMID: 18852439

    顯示更多

    收起更多

  • 相關疾病:
    Bethlem myopathy 1 (BTHLM1); Ullrich congenital muscular dystrophy 1 (UCMD1); Myosclerosis autosomal recessive (MYOSAR)
  • 亞細胞定位:
    Secreted, extracellular space, extracellular matrix. Membrane; Peripheral membrane protein. Note=Recruited on membranes by CSPG4.
  • 蛋白家族:
    Type VI collagen family
  • 數據庫鏈接:

    HGNC: 2212

    OMIM: 120240

    KEGG: hsa:1292

    STRING: 9606.ENSP00000300527

    UniGene: Hs.420269



主站蜘蛛池模板: 精品人体无码一区二区三区| 一区二区三区内射美女毛片| 国产成人精品久久亚洲高清不卡| 男人用嘴添女人私密视频| 久久无码人妻国产一区二区| 婷婷成人小说综合专区| 大白肥妇bbvbbw高潮| 欧美性受xxxx黑人xyx性爽| 五月丁香六月激情综合在线视频| 久久九九精品国产免费看小说| 日本中文字幕乱码免费| 国产综合久久久久| 久久香综合精品久久伊人| 韩国国内大量揄拍精品视频| 亚洲第一无码xxxxxx| 全黄激性性视频| 亚洲精品无码久久毛片| 国产尤物在线视精品在亚洲| 影音先锋女人av鲁色资源网久久 | 骚虎视频在线观看| 国产成人亚洲无吗淙合青草| 性xxxx欧美老妇胖老太性多毛 | 国产主播av福利精品一区| 亚洲国产欧美在线人成app| 少妇av一区二区三区无码| 日本一二免费不卡区| 国产欧美在线亚洲一区| 国产成人午夜精华液| 精品无码久久久久久国产| 亚洲欧美人成网站aaa| 免费的av网站手机版| 99久久综合精品五月天| 无遮挡激情视频国产在线观看| 日韩午夜理论片 中文字幕 | 五月天激情婷婷婷久久| 女人喷液抽搐高潮视频| 亚洲综合狠狠丁香五月| 亚洲国产成人精品无码区一本| 成年免费视频播放网站推荐| s级爆乳玩具酱国产vip皮裤 | 亚洲国产精品第一区二区三区|