NEONATAL CENTRONUCLEAR MYOPATHY WITH N-CAM DECORATED MYOTUBES

被引：8

作者：

FIDZIANSKA, A

WARLO, I

GOEBEL, HH

机构：

[1] UNIV MAINZ,DEPT NEUROPATHOL,W-6500 MAINZ,GERMANY

[2] POLISH ACAD SCI,NEUROMUSCULAR UNIT,PL-02097 WARSAW,POLAND

来源：

NEUROPEDIATRICS | 1994年 / 25卷 / 03期

关键词：

N-CAM DECORATED MYOTUBES; FAILURE IN MUSCLE MATURATION;

D O I：

10.1055/s-2008-1073015

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Muscle specimens from two baby males with fatal X-linked recessive (XLR) form of central nuclear myopathy (CNM) were studied by immunochemistry using cell surface molecules (N-CAM) and by electron microscopy. In both neonates muscle fibres with morphology of myotubes strongly expressed N-CAM. The expression of N-CAM in non regenerating postnatal human muscle suggests that neonatal X-linked fatal form of CNM is characterized by arrest or delay of maturation of muscle fibres.

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页码：158 / 161

页数：4

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