DIFFERENTIAL EXPRESSION OF MUSCULAR-DYSTROPHY IN DIAPHRAGM VERSUS HINDLIMB MUSCLES OF MDX MICE
DIFFERENTIAL EXPRESSION OF MUSCULAR-DYSTROPHY IN DIAPHRAGM VERSUS HINDLIMB MUSCLES OF MDX MICE
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DOI：
10.1002/mus.880151008
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发表时间：
1992-10-01
期刊：
影响因子：
3.4
通讯作者：
MCCARTER, RJ
中科院分区：
文献类型：
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作者：
DUPONTVERSTEEGDEN, EE;MCCARTER, RJ
本文比较了mdx小鼠和正常小鼠的横隔肌(DIA)和后肢肌肉[比目鱼肌(SOL)和指长伸肌(EDL)]的收缩特性。小鼠的年龄从2周到1.5岁不等。直接刺激肌肉，测量收缩时间、半松弛时间、单位面积主动张力、疲劳指数和最大缩短速度(V(Max))。MDX DIA组主动张力随年龄的增加而显著降低，而对照组DIA组则无明显变化。在整个年龄范围内，MDX组的SOL和EDL主动张力低于对照组，且不随年龄下降。MDX DIA组V(Max)降低，MDX Sol或EDL组V(Max)无明显变化。这些结果表明，DIA比后肢肌肉更容易受到肌肉营养不良的影响。由于许多Duchenne患者表现出呼吸窘迫，与四肢肌肉相比，隔膜营养不良的不同表现可能具有重要的临床意义。
Contractile properties of diaphragm (DIA) from mdx and control mice were compared with those of hindlimb muscles [soleus (SOL) and extensor digitorum longus (EDL)] in vitro. Mice ranged in age from 2 weeks to 1.5 years. Muscles were directly stimulated and properties measured were: contraction time, half-relaxation time, active tension per unit area, fatigue index, and maximal velocity of shortening (V(max)). Active tension decreased significantly with age in mdx DIA but not in control DIA. SOL and EDL active tensions were less in mdx than control over the whole age range and did not decrease with age. V(max) was decreased in mdx DIA, but not in mdx SOL or EDL. These results demonstrate that DIA is more affected by muscular dystrophy than hindlimb muscles. Since many Duchenne patients exhibit respiratory distress, this differential expression of dystrophy in diaphragm, as compared to limb muscles, may have important clinical implications.