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-YP005752HU
  • 規格:
  • 來源:
    Yeast
  • 其他:
  • 中文名稱:
    Recombinant Human Collagen alpha-2(VI) chain(COL6A2) ,partial
  • 貨號:
    CSB-EP005752HU-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:
    Recombinant Human Collagen alpha-2(VI) chain(COL6A2) ,partial
  • 貨號:
    CSB-BP005752HU
  • 規格:
  • 來源:
    Baculovirus
  • 其他:

產品詳情

  • 純度:
    >85% (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
  • 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:
    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?。?/div>
    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: 久久亚洲国产精品成人av秋霞 | 久久精品无码一区二区app | 中文字幕 亚洲精品 第1页| 大陆极品少妇内射aaaaa| 久久中文字幕人妻熟av女蜜柚m| 香蕉av福利精品导航| 亚洲欧美日韩在线码| 又爽又黄又无遮挡网站| 午夜高清国产拍精品| 色诱视频在线观看| 亚洲色成人网一二三区| 牲欲强的熟妇农村老妇女视频| 色综合 图片区 小说区| 青青草国产三级精品三级| 一道久在线无码加勒比| 国产精品美女久久久久久久久| 人妻人人澡人人添人人爽| 国产成人午夜福利在线观看视频| 成人女人黄网站免费视频| 国产亚洲欧美日韩俺去了 | 成人综合网站| 亚洲欧洲日产韩国在线看片| 亚洲精品av网站在线观看| 国产av无码专区亚洲版综合| 亚洲aⅴ天堂av天堂无码麻豆| 国产乱子经典视频在线观看| 热99re久久精品国产首页免费| 色欲综合久久中文字幕网| 人妻丰满熟妇av无码区| 亚洲免费人成在线视频观看| 日本又色又爽又黄的视频免 | 久久99热狠狠色精品一区| 久久人妻少妇嫩草av蜜桃| 日本理论片午午伦夜理片2021| 国产成人无码a区在线观看视频app| 亚洲精品nv久久久久久久久久| 丰满爆乳无码一区二区三区| 高潮毛片无遮挡高清免费视频| 97国产色伦在色在线播放| 国产无遮挡aaa片爽爽| 婷婷久久综合九色综合绿巨人|