Roig Manuel, Roma Josep, Fargas Arnau, Munell Francina
Secció Neurologia Infantil, Hospital Universitari Vall d'Hebron, Passeig Vall d' Hebron 119-129, 08035, Barcelona, Spain. manroig@ cs.vhebron.es
Acta Neuropathol. 2004 Jan;107(1):27-34. doi: 10.1007/s00401-003-0773-3. Epub 2003 Oct 7.
Muscle necrosis in mdx mice is rare before the 2nd week of life, but becomes pronounced from weeks 2 to 6. There is no agreement on what happens after this age. Using sequential microscopic histology, immunohistochemistry and histomorphometry, we studied the evolution of muscle pathology throughout the mdx life span. Between 2 weeks and 3 months, multiple necrotic groups involving variable numbers of fibres were observed in the same section. During this period, most necrotic groups comprised more than 15 fibres. From 3 to 6 months, necrosis diminished progressively, involving isolated fibres or groups of two to five fibres, and most fibres had a centrally located nucleus. From 6 months onward there was progressive variation of fibre size and deposition of fibrillary collagens. Mast cell counts increased progressively until 6 months of age. Diminishing necrosis rather than increasing regeneration may explain the differences in muscle pathology observed between human DMD and mouse mdx. Mast cells play a secondary role in pathogenesis.
mdx小鼠在出生后第2周之前肌肉坏死很少见，但在第2至6周会变得明显。对于这个年龄之后会发生什么尚无定论。我们使用连续显微镜组织学、免疫组织化学和组织形态计量学方法，研究了mdx小鼠整个寿命期内肌肉病理学的演变。在2周龄至3月龄之间，在同一切片中观察到多个坏死组，涉及数量不等的肌纤维。在此期间，大多数坏死组包含超过15条肌纤维。在3至6月龄时，坏死逐渐减少，涉及单个肌纤维或2至5条肌纤维的组，并且大多数肌纤维具有位于中央的细胞核。从6月龄起，肌纤维大小逐渐变化，并有纤维状胶原蛋白沉积。肥大细胞计数在6月龄前逐渐增加。坏死减少而非再生增加可能解释了人类杜氏肌营养不良症（DMD）和小鼠mdx之间观察到的肌肉病理学差异。肥大细胞在发病机制中起次要作用。
