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Matthew J Longley Selected Research

Mitochondrial Diseases (Mitochondrial Disease)

10/2023Structure-specific roles for PolG2-DNA complexes in maintenance and replication of mitochondrial DNA.
1/2023Mitochondrial DNA Enrichment for Sensitive Next-Generation Sequencing.
12/2020Polymerase γ efficiently replicates through many natural template barriers but stalls at the HSP1 quadruplex.
1/2020Single-molecule level structural dynamics of DNA unwinding by human mitochondrial Twinkle helicase.
1/2020Ultrasensitive deletion detection links mitochondrial DNA replication, disease, and aging.
10/2017Synergistic Effects of the in cis T251I and P587L Mitochondrial DNA Polymerase γ Disease Mutations.
8/2011Biochemical analysis of human POLG2 variants associated with mitochondrial disease.
9/2010Disease variants of the human mitochondrial DNA helicase encoded by C10orf2 differentially alter protein stability, nucleotide hydrolysis, and helicase activity.
8/2010Meeting report: Identification of biomarkers for early detection of mitochondrial dysfunction.
1/2009Preparation of human mitochondrial single-stranded DNA-binding protein.
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Matthew J Longley Research Topics

Disease

17Mitochondrial Diseases (Mitochondrial Disease)
10/2023 - 05/2002
7Chronic Progressive External Ophthalmoplegia (Progressive External Ophthalmoplegia)
09/2010 - 05/2002
3Diffuse Cerebral Sclerosis of Schilder (Alpers' Disease)
08/2010 - 12/2005
2Acquired Immunodeficiency Syndrome (AIDS)
05/2003 - 03/2003
1Liver Failure
01/2018
1Muscular Diseases (Myopathy)
01/2018
1Infantile onset spinocerebellar ataxia
09/2010
1Ataxia (Dyssynergia)
08/2010
1Cytochrome-c Oxidase Deficiency
06/2006
1Hypersensitivity (Allergy)
08/2003
1HIV Infections (HIV Infection)
05/2003
1Rare Diseases (Rare Disease)
01/2003

Drug/Important Bio-Agent (IBA)

15Mitochondrial DNA (mtDNA)IBA
10/2023 - 05/2002
7DNA Polymerase gammaIBA
08/2010 - 05/2002
4DNA (Deoxyribonucleic Acid)IBA
10/2023 - 05/2002
1Biomarkers (Surrogate Marker)IBA
08/2010
1Electron Transport Complex IV (Cytochrome c Oxidase)IBA
06/2006
1Phosphotransferases (Kinase)IBA
08/2003
1Mitochondrial Proteins (Mitochondrial Protein)IBA
08/2003
1CopperIBA
08/2003
1NAD (NADH)IBA
08/2003
1carbovirIBA
05/2003
12',3'-dideoxy-2',3'-didehydrothymidine monophosphateIBA
05/2003
1Antiviral Agents (Antivirals)IBA
05/2003
1NucleosidesIBA
05/2003

Therapy/Procedure

1Therapeutics
05/2003