OBJECTIVE To establish Idiopathic basal ganglia calcification(IBGC′s)disease model in mouse,and investigate the effect of Chinese herbal medicine formula SYM to prevent brain calcification caused by SLC20A2 mutations...OBJECTIVE To establish Idiopathic basal ganglia calcification(IBGC′s)disease model in mouse,and investigate the effect of Chinese herbal medicine formula SYM to prevent brain calcification caused by SLC20A2 mutations.METHODS In order to apply the IBGC model in mice,we introduced S602 W point mutation into the mouse Slc20a2.Mice(3months old)with homozygous mutation developed brain calcification as similar as IBGC in human.The SYM decoction was diluted and added into the drinking water of 2-week-old homozygous Slc20a2 KI mice for 5months.Another group of homozygous Slc20a2 KI mice were set as control and received no treatment.After 5months,to analyze the effect of the SYM decoction on brain calcification,pathological sections with the brain of Slc20a2 KI mice were made and stained.RESULTS Calcified nodules were not seen in the brain of Slc20a2 KI mice that received SYM decoction treatment,while the control group developed multiple calcifications in the thalamus region.CONCLUSION SYM decoction produced preventive effect on the formation of calcified nodules in IBGC model mice,which might be useful for the treatment of IBGC caused by SLC20A2 mutations.展开更多
基金The project supported by National Natural Science Foundation of China grants(31230045and 81271252)the National Science&Technology Pillar Program duringthe 12th Five-year Plan Period(2012BAI09B04)
文摘OBJECTIVE To establish Idiopathic basal ganglia calcification(IBGC′s)disease model in mouse,and investigate the effect of Chinese herbal medicine formula SYM to prevent brain calcification caused by SLC20A2 mutations.METHODS In order to apply the IBGC model in mice,we introduced S602 W point mutation into the mouse Slc20a2.Mice(3months old)with homozygous mutation developed brain calcification as similar as IBGC in human.The SYM decoction was diluted and added into the drinking water of 2-week-old homozygous Slc20a2 KI mice for 5months.Another group of homozygous Slc20a2 KI mice were set as control and received no treatment.After 5months,to analyze the effect of the SYM decoction on brain calcification,pathological sections with the brain of Slc20a2 KI mice were made and stained.RESULTS Calcified nodules were not seen in the brain of Slc20a2 KI mice that received SYM decoction treatment,while the control group developed multiple calcifications in the thalamus region.CONCLUSION SYM decoction produced preventive effect on the formation of calcified nodules in IBGC model mice,which might be useful for the treatment of IBGC caused by SLC20A2 mutations.
