The Specific Role of Dermatan Sulfate as an Instructive Glycosaminoglycan in Tissue Development.

ABSTRACT: The crucial roles of dermatan sulfate (DS) have been demonstrated in tissue development of the cutis, blood vessels, and bone through construction of the extracellular matrix and cell signaling. Although DS classically exerts physiological functions via interaction with collagens, growth factors, and heparin cofactor-II, new functions have been revealed through analyses of human genetic disorders as well as of knockout mice with loss of DS-synthesizing enzymes. Mutations in human genes encoding the epimerase and sulfotransferase responsible for the biosynthesis of DS chains cause connective tissue disorders including spondylodysplastic type Ehlers-Danlos syndrome, characterized by skin hyperextensibility, joint hypermobility, and tissue fragility. DS-deficient mice show perinatal lethality

SUBMITTER: Mizumoto S 

PROVIDER: S-EPMC9267682 | biostudies-literature | 2022 Jul

REPOSITORIES: biostudies-literature