Linked Life Data

8381271

Source:http://linkedlifedata.com/resource/pubmed/id/8381271

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1993-3-4
pubmed:abstractText
The pathophysiological significance of the mitochondrial microangiopathy in MELAS (mitochondrial encephalopathy, lactic acidosis, and strokelike episodes) syndrome was evaluated in an autopsy study of a nearly 13-year-old girl who had suffered from multiple infarctlike lesions in the brain, a mitochondrial myopathy-cardiomyopathy, and a generalized mitochondrial microangiopathy. Cytochemically, defects of cytochrome c oxidase (complex IV) were visualized by light and electron microscopy in the skeletal and heart muscle and in the altered vessels, as well as in single bile duct cells, with the activity of the hepatocytes being diffusely reduced, whereas in the brain, the cytochemical activity was only slightly diminished. Biochemical studies revealed a 50% reduction of both NADH (the reduced from of nicotinamide-adenine dinucleotide) dehydrogenase (complex I) and complex IV in the skeletal muscle. In the brain, complex I was diminished to 20%, whereas complex IV was only slightly below the low-normal range. Immunohistochemical studies with the use of subunit-specific antiserum samples against cytochrome c oxidase showed a varying protein profile, with loss of both mitochondrially and nuclearly derived subunits being most pronounced in the heart muscle and lesser in the skeletal muscle. In the brain, liver, bile ducts, and especially the vessels, no loss of enzyme protein content was observed. The results illustrate heterogeneous tissue expression of respiratory chain defects in MELAS syndrome and indicate that vascular cytochrome c oxidase deficiency may be involved in the cerebral manifestation of the disease, whereas in other organs like the heart, a similar pathogenetic importance of the microangiopathy cannot be verified.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0003-9985
pubmed:author
pubmed:issnType
Print
pubmed:volume
117
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
202-10
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed:year
1993
pubmed:articleTitle
Generalized mitochondrial microangiopathy and vascular cytochrome c oxidase deficiency. Occurrence in a case of MELAS syndrome with mitochondrial cardiomyopathy-myopathy and combined complex I/IV deficiency.
pubmed:affiliation
Institute for Pathology, Children's Clinic, Munich, Federal Republic of Germany.
pubmed:publicationType
Journal Article, Case Reports, Research Support, Non-U.S. Gov't