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

In Stock
  • 貨號:
    CSB-EP005752HU1
  • 規(guī)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.:
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    Partial of Isoform 2C2
  • 來源:
    E.coli
  • 分子量:
    35.2 kDa
  • 表達區(qū)域:
    254-400aa
  • 氨基酸序列
    IPGPSGPKGYRGQKGAKGNMGEPGEPGQKGRQGDPGIEGPIGFPGPKGVPGFKGEKGEFGADGRKGAPGLAGKNGTDGQKGKLGRIGPPGCKGDPGNRGPDGYPGEAGSPGERGDQGGKGDPGRPGRRGPPGEIGAKGSKGYQGNSG
    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-GST-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:
    If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
  • 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:
    3-7 business days
  • 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: 国产无遮挡又黄又爽免费视频| 成 人 免费观看网站| 国产亚洲精品一区二区三区| 精品久久久久久无码专区不卡| 少妇一夜三次一区二区| 无翼乌工口全彩肉肉无遮挡18| 亚洲另类成人小说综合网 | 特黄做受又粗又长又大又硬| 久久俺也去丁香综合色| 亚洲一卡2卡3卡4卡5卡乱码观看| 色妞www精品视频| 巨大黑人极品videos精品| 色与欲影视天天看综合网| 亚洲一区波多野结衣在线| 国产sp调教打屁股视频网站| 亚洲肥老太bbw中国熟女| 人妻在线日韩免费视频| 亚洲国产日韩欧美一区二区三区| 在线播放国产不卡免费视频| 人妻无码手机在线中文| 亚洲国产欧美在线人成最新| 色六月婷婷亚洲婷婷六月| 日韩精品无码中文字幕一区二区| 国产在不卡免费一区二区三| 男女做爰猛烈叫床视频动态图| 亚洲乱亚洲乱妇50p| 久久人妻无码中文字幕| 亚洲日韩小电影在线观看| 人人妻人人爽人人添夜夜欢视频| 7777奇米四色成人眼影| 国产精品白丝喷水在线观看| а√天堂资源中文在线官网| 日韩a片无码一区二区三区电影| 日韩人妻无码中文字幕视频| 欧美日韩精品一区二区在线播放| 337p西西人体大胆瓣开下部| 久久久国产99久久国产久麻豆| 8x国产精品视频| 久久久精品午夜免费不卡| 亚洲精品国产精品乱码不卡| 亚洲欧美成人片在线观看|