Esnous D, Edom F, Butler-Browne G S, Barbet J P
Laboratoire de Pathologie Pédiatrique, Hôpital Saint Vincent de Paul, Paris.
Bull Assoc Anat (Nancy). 1993 Dec;77(239):67-71.
Muscle biopsies were taken at the oesophageal opening and in the costal part of the diaphragm from 15 foetuses (aged between 11 and 40 weeks) and from four children aged 1 week, 5 months, 3 and 16 years. Subjects with multiple congenital abnormalities or neuromuscular diseases were not included in this study. Enzyme histochemistry (myosin ATPases) and immunohistochemistry using antibodies directed against vimentin, desmin, titin and different isoforms of the myosin heavy chains (MHCs) were carried out on serial frozen sections. The diameters of the different types of fibres were measured using a Leitz ASM 68K and the corresponding results were expressed either as mean values or as histograms. In both of the regions of the diaphragm which were examined (oesophageal opening and costal portion). The skeletal muscle fibres are formed from two successive generations in a manner very similar to that which we have previously described for the limb muscles. The chronology of the various events involved in the maturation of the diaphragm does however present certain distinctive features since the adult MHCs are expressed well before 30 weeks even though paradoxically large amounts of fetal MHC were still present at birth. These findings suggest the existence of a special regulatory mechanism in the diaphragm resulting either from its innervation by the phrenic nerve or from its specific functional constraints.
从15例胎儿(年龄在11至40周之间)以及4名年龄分别为1周、5个月、3岁和16岁的儿童身上,在食管开口处和膈肌的肋部进行了肌肉活检。患有多种先天性异常或神经肌肉疾病的受试者未纳入本研究。在连续的冰冻切片上进行了酶组织化学(肌球蛋白ATP酶)和免疫组织化学,使用了针对波形蛋白、结蛋白、肌联蛋白和肌球蛋白重链(MHCs)不同亚型的抗体。使用Leitz ASM 68K测量不同类型纤维的直径,相应结果以平均值或直方图表示。在检查的膈肌的两个区域(食管开口和肋部)。骨骼肌纤维由两代连续的纤维组成,其方式与我们之前描述的肢体肌肉非常相似。然而,膈肌成熟过程中涉及的各种事件的时间顺序确实呈现出某些独特特征,因为即使矛盾的是出生时仍存在大量胎儿MHC,但成人MHC在30周前就已大量表达。这些发现表明膈肌中存在一种特殊的调节机制,这要么是由于膈神经的支配,要么是由于其特定的功能限制。