胶原蛋白6α2抗体
规格：1尘驳/1尘濒
英文名： Collagen VI alpha 2
别名： CO6A2_HUMAN; COL6A2; COL6A2; Collagen alpha 2(VI) chain; Collagen alpha-2(VI) chain; Collagen VI alpha 2 polypeptide; PP3610.
分子量： 109kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Collagen
交叉反应：Human, Mouse, Dog, Pig, Cow, Horse,
细胞定位：细胞浆
胶原蛋白6α2抗体产物介绍：background: Collagen VI acts as a cell-binding protein. Subcellular Location: Secreted > extracellular space > extracellular matrix. Membrane. Recruited on membranes by CSPG4. Post-translational modifications: Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains. DISEASE: Defects in COL6A2 are a cause of Bethlem myopathy (BM) [MIM:158810]. BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age 胶原蛋白6α2抗体of 5) and joint contractures most frequently affecting the elbows and ankles. Defects in COL6A2 are a cause of Ullrich congenital muscular dystrophy (UCMD) [MIM:254090]; also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy. Defects in COL6A2 are the cause of myosclerosis autosomal recessive (MYOSAR) [MIM:255600]; also known 胶原蛋白6α2抗体as myosclerotic myopathy or congenital myosclerosis of Lowenthal. A condition characterized by chronic inflammation of skeletal muscle with hyperplasia of the interstitial connective tissue. The clinical picture includes slender muscles with firm 'woody' consistency and restriction of movement of many joints because of muscle contractures. Similarity: Belongs to the type VI collagen family. Contains 3 VWFA domains. Gene ID: 1292 Database links: Entrez Gene: 1292 Human Entrez Gene: 12834 Mouse Omim: 120240 Human SwissProt: P12110 Human SwissProt: Q02788 Mouse Unigene: 420269 Human Unigene: 1949 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
胶原蛋白6α2抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid