Skeletal muscle weakness in osteogenesis imperfecta mice.
Skeletal muscle weakness in osteogenesis imperfecta mice.
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DOI：
10.1016/j.matbio.2010.06.006
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发表时间：
2010-09
期刊：
影响因子：
6.9
通讯作者：
Phillips, Charlotte L.
中科院分区：
文献类型：
--
作者：
Gentry, Bettina A.;Ferreira, J. Andries;McCambridge, Amanda J.;Brown, Marybeth;Phillips, Charlotte L.
运动不耐受、肌肉疲劳和无力是成骨不全（OI）患者经常报告的、很少调查的问题。OI是一种遗传性结缔组织疾病，以骨脆性为特征，主要由前α1（I）或前α2（I）胶原基因的显性突变和最近发现的I型胶原翻译后修饰蛋白的隐性突变引起。在这项研究中，我们检查了表达轻度（+/oim）和中度重度（oim/oim）OI的小鼠的比目鱼肌（S）、跖肌（P）、腓肠肌（G）、胫骨前肌（TA）和四头肌（Q）肌肉，以寻找固有肌肉病理学的证据。特别是，肌肉重量，纤维横截面积（CSA），纤维类型，纤维组织形态学，纤维胶原蛋白含量，绝对，相对和特定的峰值强直力（分别为Po，Po/mg和Po/CSA）的个别肌肉进行了评价。Oim/oim小鼠肌肉通常较小，含有较少的纤维状胶原蛋白，Po降低，并且对于特定肌肉，在300 ms的测试持续时间内无法维持Po; +/oim小鼠具有相似但更温和的骨骼肌表型。+/OIM小鼠具有特定肌肉的轻度无力，但比它们的OIM/OIM对应物受到的影响小，OIM/OIM对应物表现出明显的骨骼肌病理。因此，oim小鼠的肌无力反映了固有的骨骼肌病理学。
Exercise intolerance, muscle fatigue and weakness are often-reported, little-investigated concerns of patients with osteogenesis imperfecta (OI). OI is a heritable connective tissue disorder hallmarked by bone fragility resulting primarily from dominant mutations in the proα1(I) or proα2(I) collagen genes and the recently discovered recessive mutations in post-translational modifying proteins of type I collagen. In this study we examined the soleus (S), plantaris (P), gastrocnemius (G), tibialis anterior (TA) and quadriceps (Q) muscles of mice expressing mild (+/oim) and moderately severe (oim/oim) OI for evidence of inherent muscle pathology. In particular, muscle weight, fiber cross-sectional area (CSA), fiber type, fiber histomorphology, fibrillar collagen content, absolute, relative and specific peak tetanic force (Po, Po/mg and Po/CSA respectively) of individual muscles were evaluated. Oim/oim mouse muscles were generally smaller, contained less fibrillar collagen, had decreased Po and an inability to sustain Po for the 300 ms testing duration for specific muscles; +/oim mice had a similar but milder skeletal muscle phenotype. +/oim mice had mild weakness of specific muscles but were less affected than their oim/oim counterparts which demonstrated readily apparent skeletal muscle pathology. Therefore muscle weakness in oim mice reflects inherent skeletal muscle pathology.
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影响因子：
4
作者：
NICHOLLS, AC;OSSE, G;POPE, FM
通讯作者：
POPE, FM
DOI：
10.1152/ajpendo.90696.2008
发表时间：
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影响因子：
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64.5
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Bram RJ