Low bone turnover and low BMD in Down syndrome: effect of intermittent PTH treatment.
Low bone turnover and low BMD in Down syndrome: effect of intermittent PTH treatment.
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DOI：
10.1371/journal.pone.0042967
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发表时间：
2012
期刊：
影响因子：
3.7
通讯作者：
Suva LJ
中科院分区：
文献类型：
--
作者：
Fowler TW;McKelvey KD;Akel NS;Vander Schilden J;Bacon AW;Bracey JW;Sowder T;Skinner RA;Swain FL;Hogue WR;Leblanc DB;Gaddy D;Wenger GR;Suva LJ
21三体几乎影响到每个器官系统，并导致唐氏综合征(DS)的复杂临床表现。差异的模式现在被认为是患者的年龄，这些模式为疾病预防和治疗带来了新的机会。低骨密度(BMD)在许多男性和女性DS患者的研究中已有报道，但21三体对骨骼的具体影响仍不清楚。因此，我们测定了小鼠DS模型Ts65Dn的骨表型和骨转换标志物。雄性Ts65Dn DS小鼠不育，表现出严重的低骨量表型，并随着年龄的增长而恶化。低骨量与成骨细胞和破骨细胞发育显著降低、骨生化标志物降低、骨形成率降低和机械强度降低相关。间歇性PTH治疗4周后，3月龄Ts65Dn小鼠观察到的低骨量明显增加。这些研究为DS的严重骨脆性提供了新的见解，并确认PTH是成人低骨量DS人群中一种潜在的合成代谢药物。
Trisomy 21 affects virtually every organ system and results in the complex clinical presentation of Down syndrome (DS). Patterns of differences are now being recognized as patients’ age and these patterns bring about new opportunities for disease prevention and treatment. Low bone mineral density (BMD) has been reported in many studies of males and females with DS yet the specific effects of trisomy 21 on the skeleton remain poorly defined. Therefore we determined the bone phenotype and measured bone turnover markers in the murine DS model Ts65Dn. Male Ts65Dn DS mice are infertile and display a profound low bone mass phenotype that deteriorates with age. The low bone mass was correlated with significantly decreased osteoblast and osteoclast development, decreased bone biochemical markers, a diminished bone formation rate and reduced mechanical strength. The low bone mass observed in 3 month old Ts65Dn mice was significantly increased after 4 weeks of intermittent PTH treatment. These studies provide novel insight into the cause of the profound bone fragility in DS and identify PTH as a potential anabolic agent in the adult low bone mass DS population.
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影响因子：
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影响因子：
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作者：
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通讯作者：
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