Abstract：
:We describe the clinical and neuropathological findings of three unrelated autopsy cases of MELAS harboring the A3243G transition in the mitochondrial DNA (mtDNA). Using immunohistochemical techniques, we studied the expression of several subunits of the respiratory chain in various brain regions from the same cases. In all three cases there was a reduced immunocytochemical staining for mtDNA-encoded subunits of the respiratory chain, confirming the presence of a defective mitochondrial protein synthesis in this disease. Mitochondrial abnormalities were mostly confined to multiple areas of different size and shape, in agreement with the focal character of the brain pathology in MELAS, and were most prominent in the cerebral cortex, providing a morphological contribution to the explanation of the cognitive regression of the patients. Immunoreactivity for mtDNA-encoded subunits was reduced in the walls of many pial and intracerebral arterioles of different brain regions but there was no clear correlation between territories of affected vessels and distribution of the histological and immunohistochemical lesions. Cerebral focal lesions in MELAS might have a metabolic nature and several pathogenetic mechanisms might be involved in the genesis of stroke-like episodes when there is a local increased ATP demand.
journal_name
Acta Neuropatholjournal_title
Acta neuropathologicaauthors
Sparaco M,Simonati A,Cavallaro T,Bartolomei L,Grauso M,Piscioli F,Morelli L,Rizzuto Ndoi
10.1007/s00401-003-0716-zsubject
Has Abstractpub_date
2003-09-01 00:00:00pages
202-12issue
3eissn
0001-6322issn
1432-0533journal_volume
106pub_type
杂志文章abstract：:N-Methyl-D-aspartate (NMDA) antagonists cause neuronal vacuolation in the posterior cingulate and retrosplenial cortex of the rat. Because the nature of neuronal pathologic changes due to NMDA antagonists may affect the potential clinical use of this class of drugs, we undertook experiments to define the nature and ti...
journal_title：Acta neuropathologica
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更新日期：2003-02-01 00:00:00
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journal_title：Acta neuropathologica
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更新日期：1986-01-01 00:00:00
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journal_title：Acta neuropathologica
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doi：10.1007/BF00690545
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journal_title：Acta neuropathologica
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更新日期：2017-03-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
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更新日期：2018-10-01 00:00:00
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journal_title：Acta neuropathologica
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doi：10.1007/BF00686619
更新日期：1987-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章,评审
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更新日期：2010-09-01 00:00:00
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更新日期：2001-01-01 00:00:00
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journal_title：Acta neuropathologica
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更新日期：1986-01-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00389493
更新日期：1994-01-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：2015-09-01 00:00:00
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journal_title：Acta neuropathologica
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journal_title：Acta neuropathologica
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更新日期：1995-01-01 00:00:00
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更新日期：1981-01-01 00:00:00
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pub_type： 杂志文章
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更新日期：1999-08-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/BF00688264
更新日期：1987-01-01 00:00:00
abstract：:The spinal cord of 20 patients with amyotrophic lateral sclerosis (ALS) and 5 patients with lower motor neuron disease (LMND) were investigated immunohistochemically using anti-human excitatory amino acid transporter 1 (EAAT1) and EAAT2 antibodies which are the astrocytic transporters. The purpose of the study was to ...
journal_title：Acta neuropathologica
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更新日期：2000-08-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
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更新日期：2001-09-01 00:00:00
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journal_title：Acta neuropathologica
pub_type： 杂志文章
doi：10.1007/s00401-004-0880-9
更新日期：2004-09-01 00:00:00
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journal_title：Acta neuropathologica
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更新日期：2002-03-01 00:00:00
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更新日期：2008-11-01 00:00:00
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journal_title：Acta neuropathologica
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更新日期：1998-12-01 00:00:00
