Metabolism of collagen in aspartylglycosaminuria: Decreased synthesis by cultured fibroblasts
- 29 September 1981
- journal article
- research article
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 5 (4) , 197-203
- https://doi.org/10.1007/bf02179141
Abstract
Decreased collagen synthesis may explain the connective tissue symptoms (e.g. skeletal deformations and susceptibility to hernias) frequently present in AGU patients. Products from the incomplete intracellular degradation of glycoproteins can interfere with collagen synthesis in AGU. Aspartylglycosaminuria might thus provide a model for studying the regulation of collagen synthesis.This publication has 23 references indexed in Scilit:
- Intracellular degradation of newly synthesized collagen is conformation‐dependentFEBS Letters, 1981
- Synthesis of hyaluronic acid and collagen in skin fibroblasts cultures from patients with osteogenesis imperfectaBiochimica et Biophysica Acta (BBA) - General Subjects, 1980
- Inhibition of procollagen cell-free synthesis by amino-terminal extension peptidesBiochemistry, 1979
- High cell density alters the ratio of type III to I collagen synthesis by fibroblastsNature, 1979
- In situ detection of mycoplasma contamination in cell cultures by fluorescent Hoechst 33258 stainExperimental Cell Research, 1977
- Structural Determination of Three Glycoasparagines Isolated from the Urine of a Patient with AspartylglycosaminuriaEuropean Journal of Biochemistry, 1976
- Quantitative Film Detection of 3H and 14C in Polyacrylamide Gels by FluorographyEuropean Journal of Biochemistry, 1975
- Defect in Conversion of Procollagen to Collagen in a Form of Ehlers-Danlos SyndromeScience, 1973
- Use of a mixture of proteinase-free collagenases for the specific assay of radioactive collagen in the presence of other proteinsBiochemistry, 1971
- Enzymatic cleavage of 2-acetamido-1-(β'-l-aspartamido)-1,2-dideoxy-β-d-glucose by human plasma and seminal fluid. Failure to detect the heterozygous state for aspartylgly-cosaminuriaClinica Chimica Acta; International Journal of Clinical Chemistry, 1969