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ITT6088

ITT6088
ITT6088
ITT6088
  • Catalog: ITT6088
  • Gene/Protein: COL8A2
  • Product Description: Immunotag™ COL8A2 Polyclonal Antibody
385.0000
Price in reward points: 385

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Immunotag™ COL8A2 Polyclonal Antibody
Antibody Specification
Datasheet
Target Protein COL8A2
Clonality Polyclonal
Storage/Stability -20°C/1 year
Application WB,ELISA
Recommended Dilution WB 1:500-2000, ELISA 1:10000-20000
Concentration 1 mg/ml
Reactive Species Human,Mouse,Rat
Host Species Rabbit
Immunogen Synthesized peptide derived from human COL8A2. at AA range: 611-660
Specificity COL8A2 Polyclonal Antibody detects endogenous levels of COL8A2
Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen
Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Gene Name COL8A2
Accession No. P25067 P25318
Alternate Names Collagen alpha-2(VIII) chain (Endothelial collagen)
Description collagen type VIII alpha 2 chain(COL8A2) Homo sapiens This gene encodes the alpha 2 chain of type VIII collagen. This protein is a major component of the basement membrane of the corneal endothelium and forms homo- or heterotrimers with alpha 1 (VIII) type collagens. Defects in this gene are associated with Fuchs endothelial corneal dystrophy and posterior polymorphous corneal dystrophy type 2. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2014],
Protein Expression PCR rescued clones,Spleen,
Subcellular Localization extracellular region,proteinaceous extracellular matrix,collagen trimer,basement membrane,endoplasmic reticulum lumen,extracellular matrix,
Protein Function disease:Defects in COL8A2 are a cause of Fuchs endothelial corneal dystrophy (FECD) [MIM:136800]. FECD is the commonest primary disorder of the corneal endothelium in developed countries. Symptoms of painful visual loss result from corneal decompensation. Signs may be present from the fourth decade of life onwards. Tipically, focal wart-like guttata arising from Descemet membrane develops in the central cornea; Descemet membrane is thickened by abnormal collagenous deposition. FECD is usually sporadic but familial highly penetrant forms showing autosomal dominant inheritance are also recognized.,disease:Defects in COL8A2 are a cause of posterior polymorphous corneal dystrophy (PPCD) [MIM:122000]. PPCD is a slowly progressive hereditary disorder of the corneal endothelium that leads to a variable degree of visual impairment usually in adulthood. PPCD is usually inherited as an autosomal dominant trait.,disease:Defects in COL8A2 are the cause of posterior polymorphous corneal dystrophy 2 (PPCD2) [MIM:609140]. PPCD is a rare bilateral familial disorder of the corneal epithelium, and is inherited in a autosomal dominant pattern. The clinical features usually present earlier than FECD, being from birth onwards. The disorder is characterized by alterations of Descemet membrane presenting as vesicles, opacities or band-like lesions on slit-lamp examination and specular microscopy. Affected patient typically are asymptomatic.,function:Major component of the Descemet membrane (basement membrane) of corneal endothelial cells.,PTM:Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.,similarity:Contains 1 C1q domain.,subunit:May form homotrimers, or heterotrimers in association with alpha 1(VIII) type collagens.,
Usage For Research Use Only! Not for diagnostic or therapeutic procedures.
Material Safety Data Sheet
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