Cellular pathology of multiple system atrophy: a review.

Lantos, Peter L.; Papp, M

doi:10.1136/jnnp.57.2.129

Cited by 133 publications

(90 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It is generally accepted that neurodegeneration does not occur in the striatal and pallidal neurons in IPD, whereas MSA-P does involve those areas. 36,40 The reason may be that imaging is only sensitive enough to demonstrate iron deposition in the putamen, where neurodegeneration occurs most.…”

Section: Discussionmentioning

confidence: 99%

Different Iron-Deposition Patterns of Multiple System Atrophy with Predominant Parkinsonism and Idiopathetic Parkinson Diseases Demonstrated by Phase-Corrected Susceptibility-Weighted Imaging

Wang¹,

Butros²,

Shuai³

et al. 2011

AJNR Am J Neuroradiol

136

122

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Different Iron-Deposition Patterns of Multiple System Atrophy with Predominant Parkinsonism and Idiopathetic Parkinson Diseases Demonstrated by Phase-Corrected Susceptibility-Weighted Imaging

Wang¹,

Butros²,

Shuai³

et al. 2011

AJNR Am J Neuroradiol

136

122

View full text Add to dashboard Cite

show abstract

“…MSA is a sporadic, progressive neurological disorder characterized by parkinsonism, cerebellar dysfunction, autonomic impairment and pyramidal signs (Graham and Oppenheimer, 1969;Gilman et al, 1999;Shults and Gilman, 2003), and ␣-syn-immunoreactive glial cytoplasmic inclusions (GCIs) in oligodendrocytes (Lantos and Papp, 1994;Lantos, 1998;Wakabayashi et al, 1998a;Dickson et al, 1999;Gai et al, 1999;Duda et al, 2000;Dickson, 2001;Goedert, 2001) in several brain regions (Wenning and Jellinger, 2005). These inclusions are accompanied by neuronal loss, astrogliosis, and demyelination (Probst-Cousin et al, 1998;Wenning and Jellinger, 2005).…”

Section: Introductionmentioning

confidence: 99%

Neurological and Neurodegenerative Alterations in a Transgenic Mouse Model Expressing Human α-Synuclein under Oligodendrocyte Promoter: Implications for Multiple System Atrophy

Shults¹,

Rockenstein²,

Crews³

et al. 2005

J. Neurosci.

219

261

View full text Add to dashboard Cite

Multiple system atrophy (MSA) is a progressive, neurodegenerative disease characterized by parkinsonism, ataxia, autonomic dysfunction, and accumulation of ␣-synuclein (␣-syn) in oligodendrocytes. To better understand the mechanisms of neurodegeneration and the role of ␣-syn accumulation in oligodendrocytes in the pathogenesis of MSA, we generated transgenic mouse lines expressing human (h) ␣-syn under the control of the murine myelin basic protein promoter. Transgenic mice expressing high levels of h␣-syn displayed severe neurological alterations and died prematurely at 6 months of age. Furthermore, mice developed progressive accumulation of h␣-synimmunoreactive inclusions in oligodendrocytes along the axonal tracts in the brainstem, basal ganglia, cerebellum, corpus callosum, and neocortex. The inclusions also reacted with antibodies against phospho-serine (129) h␣-syn and ubiquitin, and h␣-syn was found in the detergent-insoluble fraction. In high-expresser lines, the white matter tracts displayed intense astrogliosis, myelin pallor, and decreased neurofilament immunostaining. Accumulation of h␣-syn in oligodendrocytes also leads to prominent neurodegenerative changes in the neocortex with decreased dendritic density and to loss of dopaminergic fibers in the basal ganglia. The oligodendrocytic inclusions were composed of fibrils and accompanied by mitochondrial alterations and disruption of the myelin lamina in the axons. Together, these studies support the contention that accumulation of ␣-syn in oligodendrocytes promotes neurodegeneration and recapitulates several of the key functional and neuropathological features of MSA.

show abstract

“…Hence in MSA, the substantia nigra, striatum, inferior olivary nucleus, pontine nuclei, and cerebellum are affected [18,49,105]. In some cases, there may be progressive cerebral atrophy affecting the frontal lobes [103] and the motor/ premotor areas [168], the limbic system also being affected, principally in longer duration cases [138].…”

Section: Disorders: Ad -Alzheimer's Disease Agd -Argyrophilic Grain mentioning

confidence: 99%

“…In addition, a fundamental cytoskeletal alteration of oligodendrocytes occurs in MSA [18,50,170] resulting in the formation of characteristic 'glial cytoplasmic inclusions' (GCI) [135] which can be observed in the substantia nigra, striatum, inferior olivary nucleus, pontine nuclei, and cerebellum [105]. A close association between GCI and microtubules has also been demonstrated [129], aberrant or ectopic expression of cdk5 and MAPK leading to abnormal phosphorylation of microtubule cytoskeletal proteins and the formation of inclusions.…”

Section: Cellsmentioning

confidence: 99%

Can neurodegenerative disease be defined by four ‘primary determinants’: anatomy, cells, molecules, and morphology?

Armstrong

2016

View full text Add to dashboard Cite

A b s t r a c t , the anatomical pathways affected by the disease ('anatomy'), the cell populations affected ('cells'), the molecular pathology of 'signature' pathological lesions ('molecules'), and the morphological types of neurodegeneration ('morphology'). This review first discusses the limitations of existing classificatory systems and second provides evidence that the four primary determinants could be used as axes to define all cases of neurodegenerative disease.To illustrate the methodology, the primary determinants were applied to the study of a group of closely related tauopathy cases and to heterogeneity within frontotemporal lobar degeneration with .

show abstract

Cellular pathology of multiple system atrophy: a review.

Cited by 133 publications

References 19 publications

Different Iron-Deposition Patterns of Multiple System Atrophy with Predominant Parkinsonism and Idiopathetic Parkinson Diseases Demonstrated by Phase-Corrected Susceptibility-Weighted Imaging

Different Iron-Deposition Patterns of Multiple System Atrophy with Predominant Parkinsonism and Idiopathetic Parkinson Diseases Demonstrated by Phase-Corrected Susceptibility-Weighted Imaging

Neurological and Neurodegenerative Alterations in a Transgenic Mouse Model Expressing Human α-Synuclein under Oligodendrocyte Promoter: Implications for Multiple System Atrophy

Can neurodegenerative disease be defined by four ‘primary determinants’: anatomy, cells, molecules, and morphology?

Contact Info

Product

Resources

About