The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
补充说明Protein concentration is above or equal to 0.05 µg/µl
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Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.3% Glutathione, 0.79% Tris HCl
功能Major keratinocyte cell envelope protein.
疾病相关Defects in LOR are a cause of progressive symmetric erythrokeratodermia (PSEK) [MIM:133200]. Erythrokeratodermas are a group of disorders characterized by widespread erythematous plaques, either stationary or migratory, associated with features that include palmoplantar keratoderma. PSEK is characterized by erythematous and hyperkeratotic plaques. Defects in LOR are the cause of Vohwinkel syndrome with ichthyosis (VSI) [MIM:604117]; also known as loricrin keratoderma (LK) or mutilating keratoderma with ichthyosis. VSI is an ichthyotic variant of Vohwinkel syndrome (VS) characterized by progressive symmetric erythrokeratoderma or congenital ichthyosiform erythroderma born as a collodion baby. Common clinical features include hyperkeratosis of the palms and soles with digital constriction.
翻译后修饰Substrate of transglutaminases. Some glutamines and lysines are cross-linked to other loricrin molecules and to SPRRs proteins. Contains inter- or intramolecular disulfide-bonds.