A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.

The human oxidative phosphorylation (OxPhos) system consists of approximately 90 proteins encoded by nuclear and mitochondrial genomes and serves as the primary cellular pathway for ATP biosynthesis. While the core protein machinery for OxPhos is well characterized, many of its assembly, maturation,...

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Main Authors: Joshua M Baughman, Roland Nilsson, Vishal M Gohil, Daniel H Arlow, Zareen Gauhar, Vamsi K Mootha
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2009-08-01
Series:PLoS Genetics
Online Access:https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1000590&type=printable
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author Joshua M Baughman
Roland Nilsson
Vishal M Gohil
Daniel H Arlow
Zareen Gauhar
Vamsi K Mootha
author_facet Joshua M Baughman
Roland Nilsson
Vishal M Gohil
Daniel H Arlow
Zareen Gauhar
Vamsi K Mootha
author_sort Joshua M Baughman
collection DOAJ
description The human oxidative phosphorylation (OxPhos) system consists of approximately 90 proteins encoded by nuclear and mitochondrial genomes and serves as the primary cellular pathway for ATP biosynthesis. While the core protein machinery for OxPhos is well characterized, many of its assembly, maturation, and regulatory factors remain unknown. We exploited the tight transcriptional control of the genes encoding the core OxPhos machinery to identify novel regulators. We developed a computational procedure, which we call expression screening, which integrates information from thousands of microarray data sets in a principled manner to identify genes that are consistently co-expressed with a target pathway across biological contexts. We applied expression screening to predict dozens of novel regulators of OxPhos. For two candidate genes, CHCHD2 and SLIRP, we show that silencing with RNAi results in destabilization of OxPhos complexes and a marked loss of OxPhos enzymatic activity. Moreover, we show that SLIRP plays an essential role in maintaining mitochondrial-localized mRNA transcripts that encode OxPhos protein subunits. Our findings provide a catalogue of potential novel OxPhos regulators that advance our understanding of the coordination between nuclear and mitochondrial genomes for the regulation of cellular energy metabolism.
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spelling doaj-art-566fc7fd4a704dcd84fd1699d929f3a12025-08-20T03:22:38ZengPublic Library of Science (PLoS)PLoS Genetics1553-73901553-74042009-08-0158e100059010.1371/journal.pgen.1000590A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.Joshua M BaughmanRoland NilssonVishal M GohilDaniel H ArlowZareen GauharVamsi K MoothaThe human oxidative phosphorylation (OxPhos) system consists of approximately 90 proteins encoded by nuclear and mitochondrial genomes and serves as the primary cellular pathway for ATP biosynthesis. While the core protein machinery for OxPhos is well characterized, many of its assembly, maturation, and regulatory factors remain unknown. We exploited the tight transcriptional control of the genes encoding the core OxPhos machinery to identify novel regulators. We developed a computational procedure, which we call expression screening, which integrates information from thousands of microarray data sets in a principled manner to identify genes that are consistently co-expressed with a target pathway across biological contexts. We applied expression screening to predict dozens of novel regulators of OxPhos. For two candidate genes, CHCHD2 and SLIRP, we show that silencing with RNAi results in destabilization of OxPhos complexes and a marked loss of OxPhos enzymatic activity. Moreover, we show that SLIRP plays an essential role in maintaining mitochondrial-localized mRNA transcripts that encode OxPhos protein subunits. Our findings provide a catalogue of potential novel OxPhos regulators that advance our understanding of the coordination between nuclear and mitochondrial genomes for the regulation of cellular energy metabolism.https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1000590&type=printable
spellingShingle Joshua M Baughman
Roland Nilsson
Vishal M Gohil
Daniel H Arlow
Zareen Gauhar
Vamsi K Mootha
A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.
PLoS Genetics
title A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.
title_full A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.
title_fullStr A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.
title_full_unstemmed A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.
title_short A computational screen for regulators of oxidative phosphorylation implicates SLIRP in mitochondrial RNA homeostasis.
title_sort computational screen for regulators of oxidative phosphorylation implicates slirp in mitochondrial rna homeostasis
url https://journals.plos.org/plosgenetics/article/file?id=10.1371/journal.pgen.1000590&type=printable
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