Linked Life Data

760362

Source:http://linkedlifedata.com/resource/pubmed/id/760362

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1979-3-13
pubmed:abstractText
The brindled mouse (Mobr) is a neurological mutant mouse with clinical and biochemical features closely similar to Kinky hair syndrome (KHS) in humans. Neuronal degeneration in the cerebral cortex and thalamic nuclei was the constant neuropathological lesions in the CNS of the male hemizygotes of this mutant (Yajima and Suzuki, 1978). Ultrastructurally, many cortical neurons contained enlarged mitochondria with prominent tubular or vesicular cristae, which were similar to those described in the Purkinje cells in the human KHS (Ghatak et al., 1972) and in the rat brain with copper deficiency (Prohaska and Wells, 1975). Such mitochondria were observed not only in the degenerating neurons but even in the otherwise normal-appearing cortical neurons, suggesting that the mitochondrial damage possibly related to the deficient activities of the copper containing enzymes (cytochrome oxidase, etc.) preceded the neuronal degeneration. Many mitochondria in the severely degenerated neurons contained numerous electron dense spicules of possible calcium. Although rare, similar morphological alteration of neuronal mitochondria was also noted in the female heterozygotes, indicating the presence of possible subclinical defect in copper transport in the heterozygotes as well.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
0001-6322
pubmed:author
pubmed:issnType
Print
pubmed:day
12
pubmed:volume
45
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
17-25
pubmed:dateRevised
2007-11-9
pubmed:meshHeading
pubmed:year
1979
pubmed:articleTitle
Neuronal degeneration in the brain of the brindled mouse. An ultrastructural study of the cerebral cortical neurons.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S.