COL8A2 blocking peptide

COL8A2 Antibody (C-term) Blocking peptide

• Gene Names: COL8A2; FECD; PPCD; FECD1; PPCD2
• Synonyms: COL8A2; COL8A2 Antibody (C-term) Blocking peptide; Collagen alpha-2(VIII) chain; Endothelial collagen; COL8A2 blocking peptide

• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 703

• Cellular Location: Secreted, extracellular space, extracellular matrix, basement membrane
• Tissue Location: Expressed primarily in the subendothelium of large blood vessels. Also expressed in arterioles and venules in muscle, heart, kidney, spleen, umbilical cord, liver and lung and is also found in connective tissue layers around hair follicles, around nerve bundles in muscle, in the dura of the optic nerve, in cornea and sclera, and in the perichondrium of cartilaginous tissues. In the kidney, expressed in mesangial cells, glomerular endothelial cells, and tubular epithelial cells. Also expressed in mast cells, and in astrocytes during the repair process. Expressed in Descemet's membrane.
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for COL8A2 blocking peptide

Macromolecular component of the subendothelium. Major component of the Descemet's membrane (basement membrane) of corneal endothelial cells. Also component of the endothelia of blood vessels. Necessary for migration and proliferation of vascular smooth muscle cells and thus, has a potential role in the maintenance of vessel wall integrity and structure, in particular in atherogenesis (By similarity).

NCBI and Uniprot Product Information

• NCBI GI #: 45644957
• NCBI GeneID: 1296
• UniProt Accession #: P25067; Q5JV31; Q8TEJ5
• Molecular Weight: 67,244 Da
• NCBI Official Full Name: Collagen alpha-2(VIII) chain
• NCBI Official Synonym Full Names: collagen type VIII alpha 2 chain
• NCBI Official Symbol: COL8A2
• NCBI Official Synonym Symbols: FECD; PPCD; FECD1; PPCD2
• NCBI Protein Information: collagen alpha-2(VIII) chain
• UniProt Protein Name: Collagen alpha-2(VIII) chain
• UniProt Gene Name: COL8A2
• UniProt Entry Name: CO8A2_HUMAN

NCBI Description

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]

Uniprot Description

COL8A2: Macromolecular component of the subendothelium. Major component of the Descemet's membrane (basement membrane) of corneal endothelial cells. Also component of the endothelia of blood vessels. Necessary for migration and proliferation of vascular smooth muscle cells and thus, has a potential role in the maintenance of vessel wall integrity and structure, in particular in atherogenesis. Defects in COL8A2 are the cause of corneal dystrophy Fuchs endothelial type 1 (FECD1). It is an ocular disorder caused by loss of endothelium of the central cornea. It is characterized by focal wart-like guttata that arise from Descemet membrane and develop in the central cornea, epithelial blisters, reduced vision and pain. Descemet membrane is thickened by abnormal collagenous deposition. Defects in COL8A2 are the cause of posterior polymorphous corneal dystrophy type 2 (PPCD2). 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.

Protein type: Secreted, signal peptide; Secreted; Extracellular matrix

Chromosomal Location of Human Ortholog: 1p34.2

Cellular Component: endoplasmic reticulum lumen; extracellular matrix; extracellular region

Biological Process: collagen catabolic process; extracellular matrix organization and biogenesis

Disease: Corneal Dystrophy, Fuchs Endothelial, 1; Corneal Dystrophy, Posterior Polymorphous, 2