New mtDNA association model, MutPred variant load, suggests individuals with multiple mildly deleterious mtDNA variants are more likely to suffer from …

A Piotrowska-Nowak, JL Elson… - Frontiers in …, 2019 - frontiersin.org
The etiology of common complex diseases is multifactorial, involving both genetic, and
environmental factors. A role for mitochondrial dysfunction and mitochondrial DNA (mtDNA) …

Clinical and laboratory interpretation of mitochondrial mRNA variants

LJC Wong, T Chen, ES Schmitt, J Wang… - Human …, 2020 - Wiley Online Library
Abstract Interpretation of mitochondrial protein‐encoding (mt‐mRNA) variants has been
challenging due to mitochondrial characteristics that have not been addressed by American …

A broad comparative genomics approach to understanding the pathogenicity of Complex I mutations

GV Klink, H O'Keefe, A Gogna, GA Bazykin, JL Elson - Scientific reports, 2021 - nature.com
Disease caused by mutations of mitochondrial DNA (mtDNA) are highly variable in both
presentation and penetrance. Over the last 30 years, clinical recognition of this group of …

[HTML][HTML] A Comprehensive Approach to the Diagnosis of Leigh Syndrome Spectrum

MS Baldo, L Azevedo, MP Coelho, E Martins… - Diagnostics, 2024 - mdpi.com
Background: Leigh syndrome spectrum (LSS) is a novel nomenclature that encompasses
both classical Leigh syndrome and Leigh-like phenotypes. Given the heterogeneity of …

Heterologous inferential analysis (HIA) and other emerging concepts: In understanding mitochondrial variation in pathogenesis: There is no more low-hanging fruit

A Vila-Sanjurjo, PM Smith, JL Elson - Mitochondrial Medicine: Volume 3 …, 2021 - Springer
Here we summarize our latest efforts to elucidate the role of mtDNA variants affecting the
mitochondrial translation machinery, namely variants mapping to the mt-rRNA and mt-tRNA …

The role of mitochondria in ME/CFS: a perspective

C Tomas, JL Elson - Fatigue: Biomedicine, Health & Behavior, 2019 - Taylor & Francis
Chronic fatigue syndrome (CFS) also known as Myalgic encephalomyelitis (ME) is a
debilitating disease, characterized by the symptom of severe fatigue. ME/CFS is a …

Analysis of functional variants in mitochondrial DNA of Finnish athletes

J Kiiskilä, JS Moilanen, L Kytövuori, AK Niemi… - BMC genomics, 2019 - Springer
Background We have previously reported on paucity of mitochondrial DNA (mtDNA)
haplogroups J and K among Finnish endurance athletes. Here we aimed to further explore …

A novel m. 11406 T> A mutation in mitochondrial ND4 gene causes MELAS syndrome

Y Lin, X Xu, D Zhao, F Liu, Y Luo, J Du, D Wang, K Ji… - Mitochondrion, 2020 - Elsevier
Pathogenic point mutations of mitochondrial DNA (mtDNA) are associated with a large
number of heterogeneous diseases involving multiple systems with which patients may …

Mitochondrial molecular genetic results in a South African cohort: Divergent mitochondrial and nuclear DNA findings

S Meldau, EP Owen, K Khan, GT Riordan - Journal of Clinical …, 2022 - jcp.bmj.com
Aims Mitochondrial diseases form one of the largest groups of inborn errors of metabolism.
The birth prevalence is approximately 1/5000 in well-studied populations, but little has been …

What can a comparative genomics approach tell us about the pathogenicity of mtDNA mutations in human populations?

H O'Keefe, R Queen, P Lord… - Evolutionary …, 2019 - Wiley Online Library
Mitochondrial disorders are heterogeneous, showing variable presentation and penetrance.
Over the last three decades, our ability to recognize mitochondrial patients and diagnose …