货号：MAB1345

品牌：Merck Millipore

规格：200UL

目录价：￥5513.00

市场价格：￥4686.05

会员价格：￥4410.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Collagen Type VII Antibody, C-terminus, clone LH7.2 | MAB1345 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Carboxy terminal peptide of type VII collagen. View All» Epitope: C-terminus View All» Immunogen: Collagen type VII. View All» Clone: LH7.2 View All» Isotype: IgG View All» Species Reactivity: Human View All» Application Notes: Immunohistochemistry. Optimal working dilutions must be determined by end user. View All» Presentation: Ascites. Liquid with 0.1% sodium azide. View All» Storage Conditions: Maintain at -20°C in convenient aliquots for up to 12 months. Avoid repeated freeze/thaw cycles. View All» UniProt Number: Q02388 View All» Entrez Gene Number: NM_000094.3 View All» Gene Symbol:

• COL7A1

• EBR1

• EBD1

• EBDCT
  View All» Usage Statement: 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. View All» Key Applications: Immunocytochemistry View All» Entrez Gene Summary: 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. View All» UniProt Summary: 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. View All» Brand Family: Chemicon View All» Product Name: Anti-Collagen Type VII Antibody, C-terminus, clone LH7.2 | MAB1345 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 200 µL View All» Format: Ascites View All» Host: Mouse View All»
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