The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
追加情報ICC/IF: Use at an assay dependent dilution.
IHC-P: Use at a concentration of 1 - 2 µg/ml for 30 min at RT. Use acid-alcohol fixed tissues.
Staining of acid-alcohol fixed tissues requires antigen unmasking with 15,000U/ml of bovine testicular hyaluronidase in PBS, pH 7.4 for 30 min at 37oC.
IHC-Fr: Use at a concentration of 1 - 2 µg/ml for 30 min at RT.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
機能Integral component of basement membranes. Component of the glomerular basement membrane (GBM), responsible for the fixed negative electrostatic membrane charge, and which provides a barrier which is both size- and charge-selective. It serves as an attachment substrate for cells. Plays essential roles in vascularization. Critical for normal heart development and for regulating the vascular response to injury. Also required for avascular cartilage development. Endorepellin in an anti-angiogenic and anti-tumor peptide that inhibits endothelial cell migration, collagen-induced endothelial tube morphogenesis and blood vessel growth in the chorioallantoic membrane. Blocks endothelial cell adhesion to fibronectin and type I collagen. Anti-tumor agent in neovascularization. Interaction with its ligand, integrin alpha2/beta1, is required for the anti-angiogenic properties. Evokes a reduction in phosphorylation of receptor tyrosine kinases via alpha2/beta1 integrin-mediated activation of the tyrosine phosphatase, PTPN6. The LG3 peptide has anti-angiogenic properties that require binding of calcium ions for full activity.
組織特異性Found in the basement membranes.
関連疾患Defects in HSPG2 are the cause of Schwartz-Jampel syndrome (SJS1) [MIM:255800]; a rare autosomal recessive disorder characterized by permanent myotonia (prolonged failure of muscle relaxation) and skeletal dysplasia, resulting in reduced stature, kyphoscoliosis, bowing of the diaphyses and irregular epiphyses. Defects in HSPG2 are the cause of dyssegmental dysplasia Silverman-Handmaker type (DDSH) [MIM:224410]. The dyssegmental dysplasias are rare, autosomal recessive skeletal dysplasias with anisospondyly and micromelia. There are two recognized types: the severe, lethal DDSH and the milder Rolland-Desbuquois form. Individuals with DDSH also have a flat face, micrognathia, cleft palate and reduced joint mobility, and frequently have an encephalocoele. The endochondral growth plate is short, the calcospherites (which are spherical calcium-phosphorus crystals produced by hypertrophic chondrocytes) are unfused, and there is mucoid degeneration of the resting cartilage.
配列類似性Contains 4 EGF-like domains. Contains 22 Ig-like C2-type (immunoglobulin-like) domains. Contains 11 laminin EGF-like domains. Contains 3 laminin G-like domains. Contains 3 laminin IV type A domains. Contains 4 LDL-receptor class A domains. Contains 1 SEA domain.
翻訳後修飾Proteolytic processing produces the C-terminal angiogenic peptide, endorepellin. This peptide can be further processed to produce the LG3 peptide. N- and O-glycosylated; contains three heparan sulfate chains. The LG3 peptide contains at least three and up to five potential O-glycosylation sites but no N-glycosylation.
細胞内局在Secreted > extracellular space > extracellular matrix > basement membrane.
Dai L et al. Lipoteichoic Acid (LTA) and Lipopolysaccharides (LPS) from Periodontal Pathogenic Bacteria Facilitate Oncogenic Herpesvirus Infection within Primary Oral Cells. PLoS One9:e101326 (2014).
Read more (PubMed: 24971655) »
Horiguchi Y et al. Distribution, ultrastructural localization, and ontogeny of the core protein of a heparan sulfate proteoglycan in human skin and other basement membranes. J Histochem Cytochem37:961-70 (1989).
Read more (PubMed: 2659664) »