Abstract:
:Healthy nodes of Ranvier are crucial for action potential propagation along myelinated axons, both in the central and in the peripheral nervous system. Surprisingly, the node of Ranvier has often been neglected when describing CNS disorders, with most pathologies classified simply as being due to neuronal defects in the grey matter or due to oligodendrocyte damage in the white matter. However, recent studies have highlighted changes that occur in pathological conditions at the node of Ranvier, and at the associated paranodal and juxtaparanodal regions where neurons and myelinating glial cells interact. Lengthening of the node of Ranvier, failure of the electrically resistive seal between the myelin and the axon at the paranode, and retraction of myelin to expose voltage-gated K(+) channels in the juxtaparanode, may contribute to altering the function of myelinated axons in a wide range of diseases, including stroke, spinal cord injury and multiple sclerosis. Here, we review the principles by which the node of Ranvier operates and its molecular structure, and thus explain how defects at the node and paranode contribute to neurological disorders.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Arancibia-Carcamo IL,Attwell Ddoi
10.1007/s00401-014-1305-zsubject
Has Abstractpub_date
2014-08-01 00:00:00pages
161-75issue
2eissn
0001-6322issn
1432-0533journal_volume
128pub_type
杂志文章,评审abstract::Using both tumor specimen and cultured tumor cells, we have studied the differentiation of a pineocytoma by light and electron microscopy (EM) and immunohistochemical demonstration of glial, neuronal and neuroendocrine markers. Only interstitial cells were labeled with anti-glial fibrillary acidic protein and anti-S10...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010050834
更新日期:1998-05-01 00:00:00
abstract::The hemizygote of the macular mutant mice, which is clinically and neuropathologically considered to be a model of Menkes kinky hair disease (MKHD), were injected intraperitoneally four times with 10, 20, 20 and 30 micrograms of cupric chloride on days 4, 6, 8 and 10 after birth, respectively. Their cerebral and cereb...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00689595
更新日期:1988-01-01 00:00:00
abstract::Two cases of Central neurocytoma arising in the lateral ventricles are presented. Both patients had well-circumscribed masses in the right lateral ventricle causing hydrocephalus. The tumors were composed of small round cells forming Homer Wright rosettes against a fine fibrillary background. In one patient surgical r...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687981
更新日期:1986-01-01 00:00:00
abstract::Rats were given daily injections of 0.2 or 0.5 g/kg chlorophenoxyisobutyrate (clofibrate) for 9 to 46 days. Lower leg muscles were studied with light and electron microscopy. Daily treatment with 0.5 g/kg of the drug for 26--46 days caused myopathic changes. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00703319
更新日期:1977-08-16 00:00:00
abstract::Neuregulins belong to a family of multipotent growth-promoting proteins, and have been shown to have a crucial role in accumulating acetylcholine receptor at neuromuscular junctions. A functional role of neuregulins in muscle regeneration has not yet been identified. Using reverse transcription (RT)-PCR, Western blot ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-007-0198-5
更新日期:2007-04-01 00:00:00
abstract::The accumulation of calcium in the hippocampal and thalamic vascular endothelium and the perivascular space was detected histochemically by means of the pyroantimonate technique 30, 60 and 120 min after systemic kainic acid administration. An increased number of calcium pyroantimonate deposits was found in the endothe...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00685971
更新日期:1986-01-01 00:00:00
abstract::The ultrastructure of the curly fibers was examined by the transmission and immunoelectron microscopy as well as by the rapid-freeze, deep-etch and replica method. The curly fibers consisted mainly of paired helical filaments (PHF) in the neuropils, both pre- and post-synaptic. On the deep-etch replicas, PHF in the ne...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00334498
更新日期:1990-01-01 00:00:00
abstract::In an autopsy case of the sporadic juvenile ALS (a 17-year-old girl) intracytoplasmic inclusions are found in the upper and lower motor neurons and in nerve cells of the dentate nucleus, pontine nucleus, brain stem reticular formation, substantia nigra, thalamus, globus pallidus and others. Histochemically they contai...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691069
更新日期:1978-12-15 00:00:00
abstract::We have previously characterized three human leucine-rich repeats and immunoglobulin-like domains (LRIG) genes and proteins, named LRIG1-3 and proposed that they may act as suppressors of tumor growth. The LRIG1 transmembrane protein antagonizes the activity of epidermal growth factor receptor family receptor tyrosine...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-006-0032-5
更新日期:2006-03-01 00:00:00
abstract::Amebic Meningoencephalitis (AM) and Primary Amebic Meningoencephalitis (PAM) are infectious diseases essentially confined to the Central Nervous System (CNS) and caused by free-living amebas of the genus Acanthamoeba (A.) and Naegleria (N.) respectively. AM due to A. sp. (Acanthamoeba castellanii and Acanthamoeba culb...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00686877
更新日期:1977-03-31 00:00:00
abstract::Multiple different pathological protein aggregates are frequently seen in human postmortem brains and hence mixed pathology is common. Mixed dementia on the other hand is less frequent and neuropathologically should only be diagnosed if criteria for more than one full blown disease are met. We quantitatively measured ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-015-1406-3
更新日期:2015-05-01 00:00:00
abstract::Alpha-synuclein (αSyn) preformed fibrils (PFF) induce endogenous αSyn aggregation leading to reduced synaptic transmission. Neuronal activity modulates release of αSyn; however, whether neuronal activity regulates the spreading of αSyn pathology remains elusive. Here, we established a hippocampal slice culture system ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02227-6
更新日期:2020-12-01 00:00:00
abstract::Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have identified several new molecular constituents of ALS-linked cellular aggregates, including FUS, TDP-43, OPTN, UBQLN2 and the translational product...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-013-1125-6
更新日期:2013-06-01 00:00:00
abstract::The neuropathologic findings in the brain of a National Aeronautics and Space Administration (NASA) engineer, who died subsequent to nitrogen inhalation, are reported. Findings which are unusual in other forms of anoxia included relative preservation of neurons in the Sommer sector of the hippocampus and a pattern of ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00688632
更新日期:1985-01-01 00:00:00
abstract::A 79-year-old woman had a 10-year history of dementia, initially presenting as non-fluent aphasia. Magnetic resonance imaging showed frontal atrophy (left greater than right) and hyperintense foci within white matter. Neuropathologically, there was severe frontal atrophy due to cortical neuronal loss with spongy chang...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-003-0719-9
更新日期:2003-08-01 00:00:00
abstract::Mutations in the sarcomeric protein titin, encoded by TTN, are emerging as a common cause of myopathies. The diagnosis of a TTN-related myopathy is, however, often not straightforward due to clinico-pathological overlap with other myopathies and the prevalence of TTN variants in control populations. Here, we present a...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-020-02257-0
更新日期:2021-01-15 00:00:00
abstract::Pregnant rats were administered a liquid diet containing 5% (w/v) ethanol between gestational days 10 and 21, and the brains of nine offspring were examined at 8 weeks of age. Two ethanol-treated offspring showed obvious hydrocephalus characterized by markedly enlarged lateral ventricles. Most of the other ethanol-tre...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s00401-004-0901-8
更新日期:2004-11-01 00:00:00
abstract::Neurological signs were observed in 3 lambs at approximately 1 month of age, in a flock of 1 ram and 29 ewes with 43 lambs. Deterioration occurred such that the lambs had either died or been killed by 4 months of age. Necropsies of two of these lambs revealed a diffuse encephalopathy in which the most prominent featur...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00309632
更新日期:1995-01-01 00:00:00
abstract::Neurons of Area 11 in the fronto-orbital cortex of 18 unselected AIDS brains are analyzed by means of stereology. Neurological abnormalities including dementing symptoms were described in eight patients. Neuropathology diagnosed human immunodeficiency virus (HIV)-specific changes in four, and diffuse poliodystrophy in...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00294228
更新日期:1990-01-01 00:00:00
abstract::Changes in morphology, behavior of the blood-brain barrier (BBB), regional cerebral blood flow (rCBF), and local cerebral glucose utilization (LCGU) were assessed and correlated in Mongolian gerbils following 5 min cerebral ischemia, produced by bilateral clamping of the common carotid arteries. The morphological chan...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691868
更新日期:1983-01-01 00:00:00
abstract::Chronic wasting disease (CWD), a progressive neurological disorder of captive mule deer, black-tailed deer, hybrids of mule deer and white-tailed deer and Rocky Mountain elk, is characterized neuropathologically by widespread spongiform change of the neuropil, intracytoplasmic vacuolation in neuronal perikarya and ast...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00310125
更新日期:1991-01-01 00:00:00
abstract::A 40-year-old man suffering from amyotrophic lateral sclerosis with symmetrical degeneration of the thalamus and the substantia nigra is reported. The distribution pattern of the thalamic degeneration in the present case was characteristic in that the Nucleus centralis was the severest affected of the thalamic nuclei...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00691074
更新日期:1978-12-15 00:00:00
abstract::To determine the origin of the large myelinated fibers in the anterolateral funiculus (ALF) in the spinal cord of humans, myelinated fibers in the ALF of the mid-cervical spinal cord were examined quantitatively. Five groups of subjects were examined, consisting of control subjects, patients with cerebral lesions and ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/s004010051129
更新日期:1999-12-01 00:00:00
abstract::Circumscribed pink areas 0.3-1.0 cm in diameter seen in deeper parts of otherwise well-fixed brains are usually ascribed to inadequate fixation. Twenty-three patients with pink areas in their fixed brains had evidence of inflammation in at least one organ. Blood vessels in the pink areas contained bacteria. Postmortem...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00687020
更新日期:1985-01-01 00:00:00
abstract::The morphological effects of two chemically different neuroactive drugs (chlorpromazine and phenobarbital) on vasculogenesis in rat cerebellum were examined to determine the presence of vascular alterations. Therapeutic dosages of both drugs were chronically administered to separate groups of maternal rats beginning o...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692188
更新日期:1982-01-01 00:00:00
abstract::The amyloid cascade hypothesis of Alzheimer's disease (AD) is testable: it implies that interference with Abeta aggregation and plaque formation may be therapeutically useful. Abeta42 immunisation of amyloid precursor protein (APP) transgenic mice prevented plaque formation and caused removal of existing plaques. The ...
journal_title:Acta neuropathologica
pub_type: 杂志文章,评审
doi:10.1007/s00401-010-0719-5
更新日期:2010-09-01 00:00:00
abstract::Adult white rats were injected daily for 1 month with aluminum chloride and killed. Aluminum was identified, quantitated, and localized in the brains of these rats. The only detectable anomalies were crystals of aluminum and phosphorus in lysosomes and lipofuscin granules in the cytoplasm of neurons. ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00707114
更新日期:1980-01-01 00:00:00
abstract::Three weeks after inoculation of 24-day-old gnotobiotic dogs with Snyder-Hill canine distemper virus, white matter samples were taken from the primary predilection sites for canine distemper virus-associated demyelination. The plasmalogenase activity in extracts was nearly 6-fold greater than control values for a dog ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00692214
更新日期:1980-01-01 00:00:00
abstract::Two spontaneous neurodegenerative diseases of the horse, equine motor neuron disease (EMND) and equine degenerative myeloencephalopathy (EDM), have been associated with alpha-tocopherol deficiency, and both were characterized by prominent accumulations of endothelial lipopigment in the small vessels of the spinal cord...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00296510
更新日期:1995-01-01 00:00:00
abstract::The fine structures of two pineal parenchymal neoplasms has been described. The tumors contained a predominance of small, poorly differentiated cells with prominent nuclei and scanty cytoplasm. These cells were similar to those found in medulloblastomas, primitive cerebral neuroectodermal tumors, and fetal cerebellum ...
journal_title:Acta neuropathologica
pub_type: 杂志文章
doi:10.1007/BF00699239
更新日期:1981-01-01 00:00:00