Alternative Splicing and Nonsense Mediated Decay in Mitochondrial Complex-I Biogenesis and its Implication in Human Diseases
Abstract
In mammals, complex-I (NADH-ubiquinone oxidoreductase) of the mitochondrial respiratory chain has 31 supernumerary subunits in addition to the 14 conserved from prokaryotes to humans. Multiplicity of structural protein components, as well as of biogenesis factors, make complex-I a sensible pace-maker of mitochondrial respiration. The work reviewed here shows that the Alternative Splicing and Nonsense Mediated Decay pathways regulate the transcription products of different nuclear genes encoding subunits of complex I. Complex-I dysfunction has been found to be associated with several human diseases. Involvement of altered pattern of transcription products of complex-I genes in pathogenetic mechanisms of these diseases is examined
Autore Pugliese
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SARDANELLI A.M.;PAPA F.
Titolo volume/Rivista
Non Disponibile
Anno di pubblicazione
2013
ISSN
1948-593X
ISBN
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Numero di citazioni Wos
Nessuna citazione
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Numero di citazioni Scopus
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Settori ERC
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Codici ASJC
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