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The mitochondrial coenzyme Q junction and complex III: biochemistry and pathophysiology

The FEBS journal, 2022-11, Vol.289 (22), p.6936-6958

2022

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Quantification of all 12 canonical ribonucleotides by real-time fluorogenic in vitro transcription

Nucleic acids research, 2024-01, Vol.52 (1), p.e6

2024

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Mitochondrial complex III deficiency drives c-MYC overexpression and illicit cell cycle entry leading to senescence and segmental progeria

Nature communications, 2023-04, Vol.14 (1), p.2356-2356, Article 2356

2023

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MYC—an emerging player in mitochondrial diseases

Frontiers in cell and developmental biology, 2023-09, Vol.11, p.1257651-1257651

2023

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COX7A2L/SCAFI and Pre-Complex III Modify Respiratory Chain Supercomplex Formation in Different Mouse Strains with a Bcs1l Mutation

PloS one, 2016-12, Vol.11 (12), p.e0168774-e0168774

2016

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Protocol for quantification of UDP-GlcNAc using an enzymatic microplate assay

STAR protocols, 2024-03, Vol.5 (1), p.102817-102817, Article 102817

2024

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Severe neonatal MEGDHEL syndrome with a homozygous truncating mutation in SERAC1

Biochimica et biophysica acta. Molecular basis of disease, 2022-01, Vol.1868 (1), p.166298-166298, Article 166298

2022

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Exploring the Conserved Role of MANF in the Unfolded Protein Response in Drosophila melanogaster

PloS one, 2016-03, Vol.11 (3), p.e0151550-e0151550

2016

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NAD+ repletion produces no therapeutic effect in mice with respiratory chain complex III deficiency and chronic energy deprivation

The FASEB journal, 2018-11, Vol.32 (11), p.5913-5926

2018

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Alternative oxidase‐mediated respiration prevents lethal mitochondrial cardiomyopathy

EMBO molecular medicine, 2019-01, Vol.11 (1), p.n/a

2019

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Rapamycin – “One size does not fit all”

EBioMedicine, 2019-04, Vol.42, p.30-31

2019

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The Finnish genetic heritage in 2022 – from diagnosis to translational research

Disease models & mechanisms, 2022-10, Vol.15 (10)

2022

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Ret receptor tyrosine kinase sustains proliferation and tissue maturation in intestinal epithelia

The EMBO journal, 2017-10, Vol.36 (20), p.3029-3045

2017

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Ketogenic diet attenuates hepatopathy in mouse model of respiratory chain complex III deficiency caused by a Bcs1l mutation

Scientific reports, 2017-04, Vol.7 (1), p.957-16, Article 957

2017

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RNAi screening for kinases and phosphatases identifies FoxO regulators

Proceedings of the National Academy of Sciences - PNAS, 2008-09, Vol.105 (39), p.14873-14878

2008

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Enzymatic assay for UDP-GlcNAc and its application in the parallel assessment of substrate availability and protein O-GlcNAcylation

Cell reports methods, 2023-07, Vol.3 (7), p.100518-100518, Article 100518

2023

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Effect of High-Carbohydrate Diet on Plasma Metabolome in Mice with Mitochondrial Respiratory Chain Complex III Deficiency

International journal of molecular sciences, 2016-11, Vol.17 (11), p.1824-1824

2016

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Characterization of the structural and functional determinants of MANF/CDNF in Drosophila in vivo model

PloS one, 2013-09, Vol.8 (9), p.e73928-e73928

2013

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Suppression of RNAi by dsRNA-degrading RNaseIII enzymes of viruses in animals and plants

PLoS pathogens, 2015-03, Vol.11 (3), p.e1004711

2015

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Zebrafish GDNF and its co-receptor GFR[alpha]1 activate the human RET receptor and promote the survival of dopaminergic neurons in vitro.(Research Article)(glial cell-line derived neurotrophic factor)

PloS one, 2017-05, Vol.12 (5), p.e0176166

2017

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Fasting reveals largely intact systemic lipid mobilization mechanisms in respiratory chain complex III deficient mice

Biochimica et biophysica acta. Molecular basis of disease, 2020-01, Vol.1866 (1), p.165573-165573, Article 165573

2020

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Characterization of Drosophila GDNF receptor-like and evidence for its evolutionarily conserved interaction with neural cell adhesion molecule (NCAM)/FasII

PloS one, 2012-12, Vol.7 (12), p.e51997-e51997

2012

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A mouse model of mitochondrial complex III dysfunction induced by myxothiazol

Biochemical and biophysical research communications, 2014-04, Vol.446 (4), p.1079-1084

2014

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A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice

Nature communications, 2020-01, Vol.11 (1), p.322-322, Article 322

2020

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Zebrafish GDNF and its co-receptor GFRα1 activate the human RET receptor and promote the survival of dopaminergic neurons in vitro

PloS one, 2017, Vol.12 (5), p.e0176166-e0176166

2017

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The mitochondrial coenzyme Q junction and complex III: biochemistry and pathophysiology

Quantification of all 12 canonical ribonucleotides by real-time fluorogenic in vitro transcription

Mitochondrial complex III deficiency drives c-MYC overexpression and illicit cell cycle entry leading to senescence and segmental progeria

MYC—an emerging player in mitochondrial diseases

COX7A2L/SCAFI and Pre-Complex III Modify Respiratory Chain Supercomplex Formation in Different Mouse Strains with a Bcs1l Mutation

Protocol for quantification of UDP-GlcNAc using an enzymatic microplate assay

Severe neonatal MEGDHEL syndrome with a homozygous truncating mutation in SERAC1

Exploring the Conserved Role of MANF in the Unfolded Protein Response in Drosophila melanogaster

NAD+ repletion produces no therapeutic effect in mice with respiratory chain complex III deficiency and chronic energy deprivation

Alternative oxidase‐mediated respiration prevents lethal mitochondrial cardiomyopathy

Rapamycin – “One size does not fit all”

The Finnish genetic heritage in 2022 – from diagnosis to translational research

Ret receptor tyrosine kinase sustains proliferation and tissue maturation in intestinal epithelia

Ketogenic diet attenuates hepatopathy in mouse model of respiratory chain complex III deficiency caused by a Bcs1l mutation

RNAi screening for kinases and phosphatases identifies FoxO regulators

Enzymatic assay for UDP-GlcNAc and its application in the parallel assessment of substrate availability and protein O-GlcNAcylation

Effect of High-Carbohydrate Diet on Plasma Metabolome in Mice with Mitochondrial Respiratory Chain Complex III Deficiency

Characterization of the structural and functional determinants of MANF/CDNF in Drosophila in vivo model

Suppression of RNAi by dsRNA-degrading RNaseIII enzymes of viruses in animals and plants

Zebrafish GDNF and its co-receptor GFR[alpha]1 activate the human RET receptor and promote the survival of dopaminergic neurons in vitro.(Research Article)(glial cell-line derived neurotrophic factor)

Fasting reveals largely intact systemic lipid mobilization mechanisms in respiratory chain complex III deficient mice

Characterization of Drosophila GDNF receptor-like and evidence for its evolutionarily conserved interaction with neural cell adhesion molecule (NCAM)/FasII

A mouse model of mitochondrial complex III dysfunction induced by myxothiazol

A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice

Zebrafish GDNF and its co-receptor GFRα1 activate the human RET receptor and promote the survival of dopaminergic neurons in vitro

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