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Mitochondrial RNA Granules Are Centers for Posttranscriptional RNA Processing and Ribosome Biogenesis

Cell reports (Cambridge), 2015-02, Vol.10 (6), p.920-932

2015

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The Mitochondrial RNA-Binding Protein GRSF1 Localizes to RNA Granules and Is Required for Posttranscriptional Mitochondrial Gene Expression

Cell metabolism, 2013-03, Vol.17 (3), p.386-398

2013

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MITRAC Links Mitochondrial Protein Translocation to Respiratory-Chain Assembly and Translational Regulation

Cell, 2012-12, Vol.151 (7), p.1528-1541

2012

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LONP1 Is Required for Maturation of a Subset of Mitochondrial Proteins, and Its Loss Elicits an Integrated Stress Response

Molecular and cellular biology, 2018-10, Vol.38 (20)

2018

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Multi-OMICS study of a CHCHD10 variant causing ALS demonstrates metabolic rewiring and activation of endoplasmic reticulum and mitochondrial unfolded protein responses

Human molecular genetics, 2021-05, Vol.30 (8), p.687-705

2021

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BOLA3 and NFU1 link mitoribosome iron-sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome

Nucleic acids research, 2023-11, Vol.51 (21), p.11797

2023

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The mitochondrial DNA genetic bottleneck results from replication of a subpopulation of genomes

Nature genetics, 2008-12, Vol.40 (12), p.1484-1488

2008

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Reconstitution of mitochondria derived vesicle formation demonstrates selective enrichment of oxidized cargo

PloS one, 2012-12, Vol.7 (12), p.e52830-e52830

2012

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A High-Density Human Mitochondrial Proximity Interaction Network

Cell metabolism, 2020-09, Vol.32 (3), p.479-497.e9

2020

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CCDC90A (MCUR1) Is a Cytochrome c Oxidase Assembly Factor and Not a Regulator of the Mitochondrial Calcium Uniporter

Cell metabolism, 2015-01, Vol.21 (1), p.109-116

2015

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Mutations in Iron-Sulfur Cluster Scaffold Genes NFU1 and BOLA3 Cause a Fatal Deficiency of Multiple Respiratory Chain and 2-Oxoacid Dehydrogenase Enzymes

American journal of human genetics, 2011-10, Vol.89 (4), p.486-495

2011

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Mitochondrial disease therapy from thin air?

Science (American Association for the Advancement of Science), 2016-04, Vol.352 (6281), p.31-32

Shoubridge, Eric A.

2016

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SPTLC1 variants associated with ALS produce distinct sphingolipid signatures through impaired interaction with ORMDL proteins

The Journal of clinical investigation, 2022-09, Vol.132 (18)

2022

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Mutations in the mitochondrial methionyl-tRNA synthetase cause a neurodegenerative phenotype in flies and a recessive ataxia (ARSAL) in humans

PLoS biology, 2012-03, Vol.10 (3), p.e1001288-e1001288

2012

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SLC25A46 is required for mitochondrial lipid homeostasis and cristae maintenance and is responsible for Leigh syndrome

EMBO molecular medicine, 2016-09, Vol.8 (9), p.1019-1038

2016

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Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1

Human molecular genetics, 2009-06, Vol.18 (12), p.2230-2240

2009

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Mutation in TACO1 , encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome

Nature genetics, 2009-07, Vol.41 (7), p.833-837

2009

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Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism

Nature genetics, 2012-10, Vol.44 (10), p.1152-1155

2012

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Supersizing the Mitochondrial Respiratory Chain

Cell metabolism, 2012-03, Vol.15 (3), p.271-272

Shoubridge, Eric A.

2012

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Deficiency of the α Subunit of Succinate–Coenzyme A Ligase Causes Fatal Infantile Lactic Acidosis with Mitochondrial DNA Depletion

American journal of human genetics, 2007-08, Vol.81 (2), p.383-387

2007

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Radioactive labeling of mitochondrial translation products in cultured cells

Methods in molecular biology (Clifton, N.J.), 2012-01, Vol.837, p.207

2012

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Mutations in C12orf62, a Factor that Couples COX I Synthesis with Cytochrome c Oxidase Assembly, Cause Fatal Neonatal Lactic Acidosis

American journal of human genetics, 2012-01, Vol.90 (1), p.142-151

2012

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Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS‐like phenotype and Complex I deficiency independent of the mitochondrial integrated stress response

Developmental neurobiology (Hoboken, N.J.), 2023-01, Vol.83 (1-2), p.54-69

2023

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Poly (A) tail length of human mitochondrial mRNAs is tissue-specific and a mutation in LRPPRC results in transcript-specific patterns of deadenylation

Molecular genetics and metabolism reports, 2020-12, Vol.25, p.100687-100687, Article 100687

2020

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Mitochondrial RNA Granules Are Centers for Posttranscriptional RNA Processing and Ribosome Biogenesis

The Mitochondrial RNA-Binding Protein GRSF1 Localizes to RNA Granules and Is Required for Posttranscriptional Mitochondrial Gene Expression

MITRAC Links Mitochondrial Protein Translocation to Respiratory-Chain Assembly and Translational Regulation

LONP1 Is Required for Maturation of a Subset of Mitochondrial Proteins, and Its Loss Elicits an Integrated Stress Response

Multi-OMICS study of a CHCHD10 variant causing ALS demonstrates metabolic rewiring and activation of endoplasmic reticulum and mitochondrial unfolded protein responses

BOLA3 and NFU1 link mitoribosome iron-sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome

The mitochondrial DNA genetic bottleneck results from replication of a subpopulation of genomes

Reconstitution of mitochondria derived vesicle formation demonstrates selective enrichment of oxidized cargo

A High-Density Human Mitochondrial Proximity Interaction Network

CCDC90A (MCUR1) Is a Cytochrome c Oxidase Assembly Factor and Not a Regulator of the Mitochondrial Calcium Uniporter

Mutations in Iron-Sulfur Cluster Scaffold Genes NFU1 and BOLA3 Cause a Fatal Deficiency of Multiple Respiratory Chain and 2-Oxoacid Dehydrogenase Enzymes

Mitochondrial disease therapy from thin air?

SPTLC1 variants associated with ALS produce distinct sphingolipid signatures through impaired interaction with ORMDL proteins

Mutations in the mitochondrial methionyl-tRNA synthetase cause a neurodegenerative phenotype in flies and a recessive ataxia (ARSAL) in humans

SLC25A46 is required for mitochondrial lipid homeostasis and cristae maintenance and is responsible for Leigh syndrome

Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1

Mutation in TACO1 , encoding a translational activator of COX I, results in cytochrome c oxidase deficiency and late-onset Leigh syndrome

Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism

Supersizing the Mitochondrial Respiratory Chain

Deficiency of the α Subunit of Succinate–Coenzyme A Ligase Causes Fatal Infantile Lactic Acidosis with Mitochondrial DNA Depletion

Radioactive labeling of mitochondrial translation products in cultured cells

Mutations in C12orf62, a Factor that Couples COX I Synthesis with Cytochrome c Oxidase Assembly, Cause Fatal Neonatal Lactic Acidosis

Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS‐like phenotype and Complex I deficiency independent of the mitochondrial integrated stress response

The Canadian Rare Diseases Models and Mechanisms (RDMM) Network: Connecting Understudied Genes to Model Organisms

Poly (A) tail length of human mitochondrial mRNAs is tissue-specific and a mutation in LRPPRC results in transcript-specific patterns of deadenylation

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