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Proteintech
Collagen Type VI Polyclonal Antibody, CoraLite® Plus 488
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产品信息
CL488-17023
产品规格
种属反应
Mouse
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Collagen Type VI fusion protein Ag10288
偶联物
激发/发射光谱
493/522 nm
形式
Liquid
浓度
1 mg/mL
纯化类型
Antigen affinity chromatography
保存液
PBS, pH 7.3, with 0.5% BSA, 50% glycerol
内含物
0.05% ProClin 300
保存条件
-20°C, store in dark
运输条件
Wet ice
产品详细信息
Immunogen sequence: NVISCQGLA PSQGRPGLSL VKENYAELLE DAFLKNVTAQ ICIDKKCPDY TCPITFSSPA DITILLDGSA SVGSHNFDTT KRFAKRLAER FLTAGRTDPA HDVRVAVVQY SGTGQQRPER ASLQFLQNYT ALASAVDAMD FINDATDVND ALGYVTRFYR EASSGAAKKR LLLFSDGNSQ GATPAAIEKA VQEAQRAGIE IFVVVVGRQV NEPHIRVLVT GKTAEYDVAY GESHLFRVPS YQALLRGVFH QTVSRKVALG
靶标信息
This gene encodes the alpha-3 chain, one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The alpha-3 chain of type VI collagen is much larger than the alpha-1 and -2 chains. This difference in size is largely due to an increase in the number of subdomains, similar to von Willebrand Factor type A domains, that are found in the amino terminal globular domain of all the alpha chains. These domains have been shown to bind extracellular matrix proteins, an interaction that explains the importance of this collagen in organizing matrix components. Mutations in the type VI collagen genes are associated with Bethlem myopathy, a rare autosomal dominant proximal myopathy with early childhood onset. Mutations in this gene are also a cause of Ullrich congenital muscular dystrophy, also referred to as Ullrich scleroatonic muscular dystrophy, an autosomal recessive congenital myopathy that is more severe than Bethlem myopathy. Multiple transcript variants have been identified, but the full-length nature of only some of these variants has been described. [provided by RefSeq, Jun 2009].
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: CO6A3; collagen alpha 3 chain type VI; collagen alpha-3(VI) chain; collagen alpha3(VI); procollagen, type VI, alpha 3; type VI collagen alpha 3 subunit
基因别名: AI507288; Col6a-3
Entrez Gene ID: (Mouse) 12835
