Michio Hirano Selected Research

Mitochondrial Myopathies (Mitochondrial Myopathy)

1/2022	Advances in Thymidine Kinase 2 Deficiency: Clinical Aspects, Translational Progress, and Emerging Therapies.
10/2021	Synergistic Deoxynucleoside and Gene Therapies for Thymidine Kinase 2 Deficiency.
11/2019	Growth differentiation factor-15 as a biomarker of strength and recovery in survivors of acute respiratory failure.
10/2019	Advances in primary mitochondrial myopathies.
8/2019	Bioavailability and cytosolic kinases modulate response to deoxynucleoside therapy in TK2 deficiency.
4/2009	The m.3244G>A mutation in mtDNA is another cause of progressive external ophthalmoplegia.
8/2007	The myopathic form of coenzyme Q10 deficiency is caused by mutations in the electron-transferring-flavoprotein dehydrogenase (ETFDH) gene.
10/2003	Lack of paternal inheritance of muscle mitochondrial DNA in sporadic mitochondrial myopathies.
7/2003	Mitochondrial myopathy of childhood associated with mitochondrial DNA depletion and a homozygous mutation (T77M) in the TK2 gene.

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Michio Hirano Research Topics

Disease

44	Mitochondrial Diseases (Mitochondrial Disease) 01/2022 - 11/2002
26	Muscular Diseases (Myopathy) 01/2020 - 09/2002
10	Cerebellar Ataxia (Dysmetria) 11/2019 - 01/2003
9	Mitochondrial Myopathies (Mitochondrial Myopathy) 01/2022 - 07/2003
8	Chronic Progressive External Ophthalmoplegia (Progressive External Ophthalmoplegia) 10/2016 - 09/2002
8	Coenzyme Q10 Deficiency 03/2013 - 02/2006
8	Lactic Acidosis 02/2013 - 03/2005
6	MELAS Syndrome (Syndrome, MELAS) 01/2022 - 03/2005
5	Ataxia (Dyssynergia) 11/2019 - 03/2008
5	Atrophy 08/2014 - 01/2003
5	Brain Diseases (Brain Disorder) 05/2014 - 05/2007
4	Muscle Hypotonia (Hypotonia) 01/2019 - 06/2008
4	Neurodegenerative Diseases (Neurodegenerative Disease) 04/2017 - 09/2006
4	Muscle Weakness 01/2017 - 09/2012
3	Disease Progression 01/2022 - 11/2011
3	Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease) 01/2021 - 03/2008
3	Spinal Muscular Atrophy (Progressive Muscular Atrophy) 01/2021 - 01/2003
3	Dystonia (Limb Dystonia) 11/2019 - 05/2007
3	Cardiomyopathies (Cardiomyopathy) 01/2019 - 01/2017
3	Leigh Disease (Leigh's Disease) 10/2018 - 12/2006
3	Cytochrome-c Oxidase Deficiency 01/2018 - 11/2002
3	Liver Failure 01/2018 - 09/2002
3	Visceral myopathy familial external ophthalmoplegia 10/2015 - 03/2003
3	Peripheral Nervous System Diseases (PNS Diseases) 10/2015 - 09/2006
3	Diabetes Mellitus 12/2014 - 01/2005
3	Nephrotic Syndrome (Syndrome, Nephrotic) 07/2014 - 12/2006
3	Muscular Dystrophies (Muscular Dystrophy) 05/2013 - 01/2002
3	MERRF Syndrome (Myoclonic Epilepsy and Ragged Red Fibers) 09/2011 - 05/2003
3	Kearns-Sayre Syndrome 01/2007 - 09/2002
3	Mitochondrial Encephalomyopathies (Mitochondrial Encephalomyopathy) 04/2005 - 01/2004
2	Rare Diseases (Rare Disease) 10/2021 - 01/2021
2	Respiratory Insufficiency (Respiratory Failure) 11/2019 - 01/2018
2	Heart Failure 04/2017 - 01/2016
2	Deafness (Deaf Mutism) 01/2017 - 04/2002
2	Limb-Girdle Muscular Dystrophies (Limb-Girdle Muscular Dystrophy) 11/2016 - 05/2013
2	Heart Diseases (Heart Disease) 01/2016 - 05/2003
2	Neutral Lipid Storage Disease with Myopathy 09/2012 - 03/2009
2	Myoglobinuria 04/2011 - 05/2010
2	Chanarin-Dorfman Syndrome 06/2010 - 03/2009
2	Seizures (Absence Seizure) 05/2009 - 06/2008
2	Hypertrophic Cardiomyopathy (Hypertrophic Obstructive Cardiomyopathy) 05/2009 - 05/2003
2	Multiple Acyl Coenzyme A Dehydrogenase Deficiency 03/2009 - 08/2007
2	Myasthenia Gravis 01/2009 - 01/2003
2	Intestinal Pseudo-Obstruction (Intestinal Pseudoobstruction) 10/2008 - 03/2006
2	Infections 05/2007 - 09/2006
1	Ophthalmoplegia (External Ophthalmoplegia) 01/2022
1	autosomal recessive 1 Spinocerebellar ataxia 01/2021

Drug/Important Bio-Agent (IBA)

59	Mitochondrial DNA (mtDNA)IBA 01/2022 - 04/2002
19	coenzyme Q10 (CoQ10)IBA 11/2019 - 01/2003
12	Proteins (Proteins, Gene)FDA Link 01/2021 - 04/2005
11	DNA (Deoxyribonucleic Acid)IBA 01/2021 - 01/2004
7	UbiquinoneIBA 08/2012 - 03/2008
6	Electron Transport Complex IV (Cytochrome c Oxidase)IBA 08/2014 - 05/2003
6	NucleotidesIBA 05/2013 - 05/2003
5	EnzymesIBA 01/2022 - 12/2006
5	Biomarkers (Surrogate Marker)IBA 01/2020 - 09/2011
5	Mitochondrial Proteins (Mitochondrial Protein)IBA 10/2016 - 06/2005
5	Leu Transfer RNAIBA 03/2010 - 05/2003
4	ThymidineIBA 10/2021 - 05/2014
4	DeoxyuridineIBA 01/2016 - 03/2003
4	LipidsIBA 09/2012 - 08/2007
3	Lactic Acid (Lactate)FDA LinkGeneric 01/2022 - 03/2008
3	thymidine kinase 2IBA 10/2021 - 01/2011
3	CalciumIBA 01/2020 - 01/2016
3	Thymidine KinaseIBA 10/2019 - 07/2003
3	Adenosine Triphosphate (ATP)IBA 10/2018 - 07/2003
3	Thymidine PhosphorylaseIBA 12/2014 - 03/2003
3	Phospholipids (Phosphatides)FDA LinkGeneric 08/2013 - 04/2005
3	Creatine Kinase (Creatine Phosphokinase)IBA 02/2013 - 03/2010
3	Transfer RNA (tRNA)IBA 04/2011 - 05/2003
2	pyrimidineIBA 10/2021 - 10/2019
2	RNA (Ribonucleic Acid)IBA 01/2021 - 05/2003
2	2- cyclohexylidenhydrazo- 4- phenyl- thiazoleIBA 01/2021 - 03/2010
2	Phosphotransferases (Kinase)IBA 01/2021 - 03/2008
2	Sirolimus (Rapamycin)FDA Link 01/2021 - 12/2017
2	Growth Differentiation Factor 15IBA 01/2020 - 11/2019
2	Succinate Dehydrogenase (Fumarate Reductase)IBA 01/2018 - 05/2003
2	GuanineIBA 04/2017 - 05/2003
2	Adenine FDA LinkGeneric 04/2017 - 05/2003
2	triphosphoric acid (triphosphate)IBA 01/2016 - 05/2014
2	Fatty Acids (Saturated Fatty Acids)IBA 04/2011 - 03/2010
2	Cytochromes b (Cytochrome b)IBA 04/2011 - 03/2010
2	Ile Transfer RNAIBA 04/2011 - 03/2010
2	Phosphoglycerate Kinase (Kinase, Phosphoglycerate)IBA 05/2010 - 03/2010
2	TransferasesIBA 03/2010 - 02/2006
2	Acyl Coenzyme A (Acyl CoA)IBA 03/2009 - 08/2007
2	NucleosidesIBA 02/2009 - 07/2003
2	Linezolid (Zyvox)FDA Link 05/2007 - 06/2005
1	CytokinesIBA 01/2022
1	N-acetylaspartate (N-acetyl aspartate)IBA 01/2022
1	DeoxycytidineIBA 10/2021
1	Phosphatidylinositols (Phosphatidylinositol)IBA 01/2021
1	bafilomycin A1IBA 01/2021
1	Transcription Factors (Transcription Factor)IBA 01/2021
1	gamma-Aminobutyric Acid (GABA)IBA 01/2021
1	NAD (NADH)IBA 01/2021
1	Proton-Translocating ATPases (ATPase, H+)IBA 01/2021
1	ExoribonucleasesIBA 01/2021

Therapy/Procedure

17	Therapeutics 10/2021 - 01/2003
2	Stem Cell Transplantation 10/2015 - 11/2011