Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: EC:3.1.6.4 (
chondroitinase
)
2,039
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Changes in the structure and organization of collagen fibrils were recently described in the skin of
aspartylglycosaminuria
patients. The skin of the patients contained a normal amount and distribution of glycosaminoglycans, but the dermatan sulfate of
aspartylglycosaminuria
skin was more sensitive to chondroitinase AC digestion, resulting in unsaturated 4-sulfated disaccharides which were not detected in controls. Isolated dermatan sulfate chains as well as the chains present in the intact core protein synthesized by skin fibroblasts from an
aspartylglycosaminuria
patient were also digestible with chondroitinase AC, while those of a control fibroblast culture could be digested with
chondroitinase
ABC only. This is indirect evidence for abnormal epimerization of dermatan sulfate in the skin of
aspartylglycosaminuria
patients, which may be associated with the changes in collagen fibril formation.
...
PMID:Abnormal dermal proteoglycan in aspartylglycosaminuria: a possible mechanism for ultrastructural changes of collagen fibrils in a glycoprotein storage disorder. 313 50
