Brachymorphic mice (bm/bm): A generalized biochemical defect expressed primarily in cartilage
[摘要] Homozygousbrachymorphic(bm/bm)micehaveadisproportionatelyshortstature.Previousstudieshaveshownthatthecartilageproteoglycanisundersulfatedasaresultofdecreased3′-phosphoadenosine5′-phosphosulfate(PAPS)levels.Inthestudiesreportedhere,PAPSsynthesizingactivitywasfoundtobedecreasedinbothskinfibroblastsandprechondrogenicmesenchyme,butsulfationofglycosaminoglycanwasnormalinthosetissuesunlessglycosaminoglycansynthesiswasenhancedbyβ-d-xyloside.Furthermore,undersulfationwascorrelatedwithincreasedproteoglycansynthesisasthelimbmesenchymeculturesunderwentchondrogenesis,andsulfationproceededinan“allornoneâ€manner.Theseobservationsdemonstratethatthemoleculardefectinbm/bmmiceisnotrestrictedtocartilage,butismanifestedtherebecauseofthelargeamountofchondroitinsulfatesynthesized.
[发布日期] [发布机构]
[效力级别] [学科分类] 生物科学（综合）
[关键词] [时效性]
