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merck millipore,默克密理博,MAB2500,Anti-Collagen Type VII Antibody, clone 32,-VII
产品名称：Anti-Collagen Type VII Antibody, clone 32,-VII
产品型号：MAB2500
Anti-Collagen Type VII Antibody, clone 32, -VII detects level of Collagen Type VII & has been published & validated for use in ELISA.
merck millipore,默克密理博,MAB2500,Anti-Collagen Type VII Antibody, clone 32,-VII
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H, B ELISA M Purified Monoclonal Antibody 描述 产品目录编号 MAB2500 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Collagen Type VII Antibody, clone 32,-VII 产品信息 格式 Purified 演示 Liquid at 1 mg/mL in 20 mM phosphate buffer, 250 mM NaCl, pH 7.6, containing 0.1% sodium azide. 应用 应用 Anti-Collagen Type VII Antibody, clone 32, -VII detects level of Collagen Type VII & has been published & validated for use in ELISA. 主要应用 - ELISA
应用说明 ELISA
IH on unfixed, weakly fixed tissue sections.生物信息 克隆 32, mAb-VII 宿主 Mouse 特异性 Monoclonal antibody MAB2500 recognizes human and bovine Type VII Collagen. 同种型 IgG1 品种反应性 HumanBovine 抗体类型 Monoclonal Antibody Entrez基因编号 - NM_000094.3
Entrez基因汇总 This gene encodes the alpha chain of type VII collagen. The type VII collagen fibril, composed of three identical alpha collagen chains, is restricted to the basement zone beneath stratified squamous epithelia. It functions as an anchoring fibril between the external epithelia and the underlying stroma. Mutations in this gene are associated with all forms of dystrophic epidermolysis bullosa. In the absence of mutations, however, an acquired form of this disease can result from an autoimmune response made to type VII collagen. 基因符号 - EBDCT
- COL7A1
- EBR1
- EBD1
UniProt编号 - Q02388
UniProt汇总 FUNCTION: SwissProt: Q02388 # Stratified squamous epithelial basement membrane protein that forms anchoring fibrils which may contribute to epithelial basement membrane organization and adherence by interacting with extracellular matrix (ECM) proteins such as type IV collagen.
SIZE: 2944 amino acids; 295220 Da
SUBUNIT: Homotrimer.
SUBCELLULAR LOCATION: Secreted, extracellular space, extracellular matrix, basement membrane.
PTM: Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
DISEASE: SwissProt: Q02388 # Epidermolysis bullosa acquisita (EBA) is an autoimmune acquired blistering skin disease resulting from autoantibodies to type VII collagen. & Defects in COL7A1 are the cause of dystrophic epidermolysis bullosa (DEB) [MIM:131750, 226600]. DEB is a group of blistering skin diseases with either autosomal dominant or autosomal recessive inheritance. Ultrastructurally, DEB is characterized by tissue separation which occurs below the dermal- epidermal basement membrane at the level of the anchoring fibrils. Various clinical types with different severity are recognized. DEB Pasini type is a severe, dominantly inherited form. Among the recessively inherited forms, the Hallopeau-Siemens type epidermolysis bullosa is the most severe form. It manifests with mutilating scarring, joint contractures, corneal erosions, esophagus structures, and propensity to formation of cutaneous squamous cell carcinomas leading to premature demise of the affected individuals. Two milder recessive forms are dystrophic epidermolysis bullosa mitis type and the localized type. The mitis type shows lifelong blistering tendency, with limited scarring and less frequent extracutaneous manifestations. In the localized type, blistering and scarring are predominantly localized to the extremities. & Defects in COL7A1 are the cause of transient bullous dermolysis of the newborn (TBDN) [MIM:131705]. TBDN is a neonatal skin blistering disorder with features similar to those observed in dystrophic epidermolysis bullosa. TBDN is characterized by sub- epidermal blisters, reduced or abnormal anchoring fibrils at the dermo-epidermal junction, and electron-dense inclusions in keratinocytes. TBDN heals spontaneously or strongly improves within the first months and years of life. & Defects in COL7A1 are the cause of pretibial type dystrophic epidermolysis bullosa (P-DEB) [MIM:131850]. Inheritance is autosomal dominant. & Defects in COL7A1 are the cause of Bart type dystrophic epidermolysis bullosa (B-DEB) [MIM:132000]; also known as epidermolysis bullosa with congenital localized absence of skin and deformity of nails. Inheritance is autosomal dominant. & Defects in COL7A1 are the cause of epidermolysis bullosa pruriginosa (EBP) [MIM:604129]. EBP is a distinct clinical subtype of DEB. It is characterized by skin fragility, blistering, scar formation, intense pruritus and excoriated prurigo nodules. Onset is in early childhood, but in some cases is delayed until the second or third decade of life. Inheritance can be autosomal dominant or recessive. & Defects in COL7A1 are the cause of isolated toenail dystrophy without skin fragility [MIM:607523]. & Defects in COL7A1 are the cause of epidermolysis bullosa dystrophica with subcorneal cleavage (EBDSC) [MIM:607600]; also known as epidermolysis bullosa simplex superficialis (EBSS). EBDSC is a new variant of epidermolysis bullosa simplex (EBS), characterized by the development of skin cleavage just beneath the level of stratum corneum. It appears to be transmitted as an autosomal dominant trait and differs from other autosomal dominant forms of EBS by the common findings of milia and atrophic scarring, as well as involvement of oral and/or ocular surfaces. It is further differentiated by the presence of blisters and the absence of spontaneous continual exfoliation or peeling.
SIMILARITY: SwissProt: Q02388 ## Contains 1 BPTI/Kunitz inhibitor domain. & Contains 9 fibronectin type-III domains. & Contains 2 VWFA domains.产品使用声明 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain refrigerated at 2-8°C in undiluted aliquots for up to 12 months. 包装信息 数量 100 µg
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