Distinct ankyrin isoforms at neuron cell bodies and nodes of Ranvier resolved using erythrocyte ankyrin-deficient mice.
Distinct ankyrin isoforms at neuron cell bodies and nodes of Ranvier resolved using erythrocyte ankyrin-deficient mice.
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DOI：
10.1083/jcb.114.6.1243
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发表时间：
1991-09
期刊：
影响因子：
--
通讯作者：
Bennett V
中科院分区：
文献类型：
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作者：
Kordeli E;Bennett V
与红细胞锚定蛋白（ankyrinRo）免疫相关的锚定蛋白（ankyrinsR）异构体与具有重要功能的不同神经元质膜结构域相关，如细胞体和树突、轴突丘和初始节段以及Ranvier淋巴结。AnkyrinRo在大脑中表达，并且至少占ankyrinR亚型的一种。另一种脑锚蛋白异构体，锚蛋白b，由一种独特的基因编码，在免疫学上与锚蛋白r不同。正常母细胞病的突变小鼠（nb/nb）构成了第一个描述的锚蛋白缺缺症的遗传模型：由于网织红细胞和大脑中锚蛋白ro基因的表达显著降低，它们表现出严重的溶血性贫血（Peters l.l., c.s. Birkenmeier, r.t. Bronson, r.a. White, s.e. Lux, e.o toto， V. Bennett, a . Higgins，和j.e. Barker. 1991）。[j] .细胞生物学杂志。114:1233- 1241。在本报告中，我们通过免疫印迹分析和免疫荧光定位nb/nb和正常小鼠的整个神经系统（前脑、小脑、脑干、脊髓和坐骨神经）的ankyrinR，来区分ankyrinRo和其他ankyrinR亚型。这是nb突变的神经系统成分的第一个免疫细胞化学特征，显示如下。(a) nb/nb小鼠在Ranvier节点和初始轴突段存在锚蛋白同型体，且不与锚蛋白特异性抗体交叉反应；因此，这种异构体不同于在大脑中发现的两种锚蛋白异构体，ankyrinRo和ankyrinB，可能是一个独特基因的产物，是与Ranvier淋巴结相关的特化膜骨架的独特成分。(b) nb/nb小鼠中缺失的AnkyrinRo选择性地与神经元细胞体和树突相关，排除在髓鞘轴突外，在神经系统中表现出选择性表达模式，在小脑神经元（浦肯野细胞和颗粒细胞）和脊髓中几乎普遍表达，并且仅限于海马和前脑新皮层的极少数神经元。
Isoforms of ankyrin (ankyrinsR) immunologically related to erythrocyte ankyrin (ankyrinRo) are associated with distinct neuronal plasma membrane domains of functional importance, such as cell bodies and dendrites, axonal hillock and initial segments, and nodes of Ranvier. AnkyrinRo is expressed in brain, and accounts for at least one of the ankyrinR isoforms. Another ankyrin isoform of brain, ankyrinB, is encoded by a distinct gene and is immunologically distinct from ankyrinsR. Mutant mice with normoblastosis (nb/nb) constitute the first described genetic model of ankyrin deficiency: they display a severe hemolytic anemia due to a significantly reduced expression of the ankyrinRo gene in reticulocytes as well as brain (Peters L. L., C. S. Birkenmeier, R. T. Bronson, R. A. White, S. E. Lux, E. Otto, V. Bennett, A. Higgins, and J. E. Barker. 1991. J. Cell Biol. 114:1233- 1241). In the present report, we distinguish between ankyrinRo and other ankyrinR isoforms using immunoblot analysis and immunofluorescence localization of ankyrinsR throughout the nervous system (forebrain, cerebellum, brain stem, spinal cord, and sciatic nerve) of nb/nb and normal mice. This is the first immunocytochemical characterization of the neurological component of the nb mutation and shows the following. (a) The isoform of ankyrin at the nodes of Ranvier and initial axonal segments is present in the nb/nb mice and does not cross-react with an ankyrinRo-specific antibody; this isoform, therefore, is distinct from both ankyrin isoforms identified in brain, ankyrinRo and ankyrinB, and is probably the product of a distinct gene and a unique component of the specialized membrane skeleton associated with nodes of Ranvier. (b) AnkyrinRo missing from nb/nb mice is selectively associated with neuronal cell bodies and dendrites, excluded from myelinated axons, and displays a selective pattern of expression in the nervous system whereby expression is almost ubiquitous in neurons of the cerebellum (Purkinje and granule cells) and spinal cord, and restricted to a very minor subset of neurons in hippocampus and neocortex of forebrain.