Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
11
pubmed:dateCreated
1993-9-16
pubmed:abstractText
cDNA libraries constructed from cytoplasmic RNA of normal and xeroderma pigmentosum (XP) fibroblast strains were screened for differential gene expression. XP fibroblast strains included one representative of the complementation groups A, C, D, and one XP variant strain. The XP lambda gt10 cDNA libraries were differentially screened with in vitro transcripts made from cDNA in the pBluescript vector using both the same XP strain and the normal fibroblast strain. Eight differential clones were detected in the libraries of the XP group A, D, and C strains, which caused stronger signals when probed with transcripts from XP strains than with those from the normal strain. The cDNA clones were sequenced. Seven of the eight clones detected coded for three mitochondrial genes: subunit I of cytochrome c oxidase (complex IV of the respiratory chain), apocytochrome b (subunit of complex III), and 16-S rRNA. Two clones representing essentially (a) subunit I of cytochrome c oxidase and (b) 16-S rRNA diverged from the sequence of the human mitochondrial genome present in the data-base libraries. Clone a exhibited a transition mutation, clone b reflected a transcript of a mitochondrial genome rearranged in the 16-S rRNA gene, including four nucleotides of the adjacent tRNA(Leu) gene. The apparently enhanced expression of mitochondrial genes in XP cells, together with the changes in DNA sequence, seem to indicate that functions of the ATP-generating system were impaired. This defect may have originated from mutations due to lack of DNA repair. The data can be interpreted in the light of mitochondrial changes that cause human neuromyopathies to occur. In analogy to these diseases the neurological symptoms in XP might be explained by abnormal mitochondria.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0171-5216
pubmed:author
pubmed:issnType
Print
pubmed:volume
119
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
675-84
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed-meshheading:8394367-Adenosine Triphosphate, pubmed-meshheading:8394367-Adolescent, pubmed-meshheading:8394367-Adult, pubmed-meshheading:8394367-Apoproteins, pubmed-meshheading:8394367-Base Sequence, pubmed-meshheading:8394367-Cloning, Molecular, pubmed-meshheading:8394367-Cytochrome b Group, pubmed-meshheading:8394367-Cytochromes b, pubmed-meshheading:8394367-DNA, Mitochondrial, pubmed-meshheading:8394367-DNA, Recombinant, pubmed-meshheading:8394367-DNA Damage, pubmed-meshheading:8394367-DNA Mutational Analysis, pubmed-meshheading:8394367-Electron Transport Complex IV, pubmed-meshheading:8394367-Female, pubmed-meshheading:8394367-Fibroblasts, pubmed-meshheading:8394367-Gene Expression, pubmed-meshheading:8394367-Genetic Complementation Test, pubmed-meshheading:8394367-Genetic Vectors, pubmed-meshheading:8394367-Genomic Library, pubmed-meshheading:8394367-Humans, pubmed-meshheading:8394367-Male, pubmed-meshheading:8394367-Mitochondria, Muscle, pubmed-meshheading:8394367-Molecular Sequence Data, pubmed-meshheading:8394367-Multigene Family, pubmed-meshheading:8394367-Pregnancy-Specific beta 1-Glycoproteins, pubmed-meshheading:8394367-RNA, Ribosomal, 16S, pubmed-meshheading:8394367-Sequence Alignment, pubmed-meshheading:8394367-Sequence Homology, Nucleic Acid, pubmed-meshheading:8394367-Xeroderma Pigmentosum
pubmed:year
1993
pubmed:articleTitle
Enhanced expression of mitochondrial genes in xeroderma pigmentosum fibroblast strains from various complementation groups.
pubmed:affiliation
Division of Interaction of Carcinogens with Biological Macromolecules, German Cancer Research Center, Heidelberg.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't