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韦弗小鼠小脑的区域化缺陷。

Regionalization defects in the weaver mouse cerebellum.

作者信息

Eisenman L M, Gallagher E, Hawkes R

机构信息

Department of Pathology, Anatomy, and Cell Biology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania 19107-6799, USA.

出版信息

J Comp Neurol. 1998 May 18;394(4):431-44.

PMID:9590553
Abstract

The mammalian cerebellum consists of parasagittal bands and transverse zones that are laid down early in development. When the adult cerebellum is immunostained for the Purkinje cell-specific antigen zebrin II (i.e., aldolase C), compartmentation is reflected in alternating zebrin II+ (P+) and zebrin II- bands (P ). The zebrin II phenotype is Purkinje cell autonomous; thus, disruptions in the zebrin pattern may reflect early problems in pattern formation. Zebrin II expression has been examined in the weaver (wv) mouse cerebellum. Both zebrin II- and zebrin II Purkinje cells are present in the homozygous weaver (wv/wv) mouse, but they are not distributed normally. In the posterior vermis, although the zebrin II+ bands are wider and multilaminate, the standard compartmentation is present. However, a large zebrin II+ cell mass is absent from the central vermis, and analysis of the anterior lobe reveals several missing zebrin II- bands. The cytoarchitectonic defects in wv mice are not simply related to the Purkinje cell abnormalities. Instead, serial reconstruction reveals two transverse boundaries-one rostrally in lobule VI and the other caudally in lobule IX-that delineate cytoarchitectonic transverse zones important in cerebellar development. The abnormal zebrin expression pattern in wv/wv mice may be secondary to the deletion of a transverse zone. This is the first demonstration that Purkinje cell compartmentation can be altered by mutation; therefore, the wv mutation should prove valuable in understanding cerebellar regionalization.

摘要

哺乳动物的小脑由矢状旁带和横向区带组成,这些结构在发育早期就已形成。当用浦肯野细胞特异性抗原zebrin II(即醛缩酶C)对成年小脑进行免疫染色时,分区表现为zebrin II阳性(P+)和zebrin II阴性带(P-)交替出现。zebrin II表型是浦肯野细胞自主的;因此,zebrin模式的破坏可能反映了早期模式形成中的问题。已在weaver(wv)小鼠小脑中检测了zebrin II的表达。纯合weaver(wv/wv)小鼠中同时存在zebrin II阴性和zebrin II阳性的浦肯野细胞,但它们的分布不正常。在小脑蚓部后部,虽然zebrin II阳性带更宽且呈多层状,但标准的分区仍然存在。然而,小脑蚓部中央没有一个大的zebrin II阳性细胞团,对前叶的分析显示有几条缺失的zebrin II阴性带。wv小鼠的细胞构筑缺陷不仅仅与浦肯野细胞异常有关。相反,连续重建显示出两个横向边界——一个在小叶VI的前部,另一个在小叶IX的后部——它们划定了在小脑发育中重要的细胞构筑横向区带。wv/wv小鼠中异常的zebrin表达模式可能是横向区带缺失的继发结果。这是首次证明浦肯野细胞分区可因突变而改变;因此,wv突变在理解小脑区域化方面应具有重要价值。

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