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Anti-COL7A1 Antibody

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产品名称: Anti-COL7A1 Antibody
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Anti-COL7A1 Antibody


Anti-COL7A1 Antibody  的详细介绍
Name: Anti-COL7A1 Antibody
See all COL7A1 primary antibodies
Description: Rabbit polyclonal antibody to COL7A1
Specificity: The antibody detects endogenous levels of total COL7A1 protein.
Applications: IHC
Reactivity: Human
Immunogen: Synthetic peptide corresponding to residues near the C terminal of human collagen, type VII, alpha 1
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: Antigen affinity purification.
Concentration: 2mg / ml
Formulation: Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
Storage: Store at -20?C
Function: 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.
Involvement in Disease: Epidermolysis bullosa dystrophica, autosomal dominant: A group of autosomal dominant blistering skin diseases 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, ranging from severe mutilating forms to mild forms with limited and localized scarring, and less frequent extracutaneous manifestations.

Epidermolysis bullosa dystrophica, autosomal recessive: A group of autosomal recessive blistering skin diseases 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, ranging from severe mutilating forms to mild forms with limited and localized scarring, and less frequent extracutaneous manifestations. Mild forms include epidermolysis bullosa mitis and epidermolysis bullosa localisata.

Epidermolysis bullosa dystrophica, Pasini type: A severe, dominantly inherited form of dystrophic epidermolysis bullosa characterized by albopapuloid Pasini papule, dorsal extremity blistering, milia formation and red atrophic scarring after recurrent blisters.

Epidermolysis bullosa dystrophica, Hallopeau-Siemens type: The most severe recessive form of dystrophic epidermolysis bullosa. 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.

Transient bullous dermolysis of the newborn: TBDN is a neonatal form of dystrophic epidermolysis bullosa 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.

Epidermolysis bullosa dystrophica, pretibial type: A form of dystrophic epidermolysis bullosa characterized by pretibial blisters that develop into prurigo-like hyperkeratotic lesions. It predominantly affects the pretibial areas, sparing the knees and other parts of the skin. Other clinical features include nail dystrophy, albopapuloid skin lesions, and hypertrophic scars without pretibial predominance. The phenotype shows considerable interindividual variability. Inheritance is autosomal dominant.

Epidermolysis bullosa dystrophica, Bart type: An autosomal dominant form of dystrophic epidermolysis bullosa characterized by congenital localized absence of skin, skin fragility and deformity of nails.

Epidermolysis bullosa pruriginosa: A distinct clinical subtype of epidermolysis bullosa dystrophica. 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.

Nail disorder, non-syndromic congenital, 8: A nail disorder characterized by isolated toenail dystrophy. The nail changes are most severe in the great toes and consist of the nail plate being buried in the nail bed with a deformed and narrow free edge.

Epidermolysis bullosa dystrophica, with subcorneal cleavage: A bullous skin disorder with variable sized clefts just beneath the level of the stratum corneum. Clinical features include blisters, milia, atrophic scarring, nail dystrophy, and oral and conjunctival involvement, as seen in dystrophic epidermolysis bullosa.
Post-Translational Modification: Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
Cellular Location: Secreted > Extracellular space > Extracellular matrix > Basement membrane.
Database Links:
  • Entrez Gene: 1294?Human
  • Omim: 120120?Human
  • SwissProt: Q02388?Human
  • Unigene: 476218?Human
    		•
    Synonyms:
  • Alpha 1 type VII collagen Antibody
  • CO7A1_HUMAN Antibody
  • Col7a1 Antibody
  • Collagen alpha 1(VII) chain Antibody
  • Collagen alpha-1(VII) chain Antibody
  • Collagen type VII alpha 1 Antibody
  • Collagen VII Antibody
  • Collagen VII alpha 1 polypeptide Antibody
  • CollagenVII Antibody
  • EBD 1 Antibody
  • EBD1 Antibody
  • EBDCT Antibody
  • EBR 1 Antibody
  • EBR1 Antibody
  • LC collagen Antibody
  • Long chain collagen Antibody
  • Long-chain collagen Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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