Targeting the biology of aging with mTOR inhibitors
JB Mannick, DW Lamming - Nature Aging, 2023 - nature.com
Inhibition of the protein kinase mechanistic target of rapamycin (mTOR) with the Food and
Drug Administration (FDA)-approved therapeutic rapamycin promotes health and longevity …
Drug Administration (FDA)-approved therapeutic rapamycin promotes health and longevity …
mTORC2: The other mTOR in autophagy regulation
J Ballesteros‐Álvarez, JK Andersen - Aging cell, 2021 - Wiley Online Library
The mechanistic target of rapamycin (mTOR) has gathered significant attention as a
ubiquitously expressed multimeric kinase with key implications for cell growth, proliferation …
ubiquitously expressed multimeric kinase with key implications for cell growth, proliferation …
Leigh syndrome: a tale of two genomes
AB Bakare, EJ Lesnefsky, S Iyer - Frontiers in Physiology, 2021 - frontiersin.org
Leigh syndrome is a rare, complex, and incurable early onset (typically infant or early
childhood) mitochondrial disorder with both phenotypic and genetic heterogeneity. The …
childhood) mitochondrial disorder with both phenotypic and genetic heterogeneity. The …
Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention
MAE Van De Wal, MJW Adjobo-Hermans, J Keijer… - Brain, 2022 - academic.oup.com
Mitochondria are small cellular constituents that generate cellular energy (ATP) by oxidative
phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous …
phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous …
[HTML][HTML] Leukocytes mediate disease pathogenesis in the Ndufs4 (KO) mouse model of Leigh syndrome
JC Stokes, RL Bornstein, K James, KY Park… - JCI insight, 2022 - ncbi.nlm.nih.gov
Symmetric, progressive, necrotizing lesions in the brainstem are a defining feature of Leigh
syndrome (LS). A mechanistic understanding of the pathogenesis of these lesions has been …
syndrome (LS). A mechanistic understanding of the pathogenesis of these lesions has been …
The immune system as a driver of mitochondrial disease pathogenesis: a review of evidence
A Hanaford, SC Johnson - Orphanet Journal of Rare Diseases, 2022 - Springer
Background Genetic mitochondrial diseases represent a significant challenge to human
health. These diseases are extraordinarily heterogeneous in clinical presentation and …
health. These diseases are extraordinarily heterogeneous in clinical presentation and …
Cannabidiol ameliorates mitochondrial disease via PPARγ activation in preclinical models
E Puighermanal, M Luna-Sánchez, A Gella… - Nature …, 2024 - nature.com
Mutations in mitochondrial energy-producing genes lead to a heterogeneous group of
untreatable disorders known as primary mitochondrial diseases (MD). Leigh syndrome (LS) …
untreatable disorders known as primary mitochondrial diseases (MD). Leigh syndrome (LS) …
Iron status influences mitochondrial disease progression in Complex I-deficient mice
CJ Kelly, RK Couch, VT Ha, CM Bodart, J Wu, S Huff… - Elife, 2023 - elifesciences.org
Mitochondrial dysfunction caused by aberrant Complex I assembly and reduced activity of
the electron transport chain is pathogenic in many genetic and age-related diseases. Mice …
the electron transport chain is pathogenic in many genetic and age-related diseases. Mice …
The extensive and functionally uncharacterized mitochondrial phosphoproteome
NM Niemi, DJ Pagliarini - Journal of Biological Chemistry, 2021 - ASBMB
More than half a century ago, reversible protein phosphorylation was linked to mitochondrial
metabolism through the regulation of pyruvate dehydrogenase. Since this discovery, the …
metabolism through the regulation of pyruvate dehydrogenase. Since this discovery, the …
Mitochondrial augmentation of CD34+ cells from healthy donors and patients with mitochondrial DNA disorders confers functional benefit
E Jacoby, M Ben Yakir-Blumkin… - NPJ Regenerative …, 2021 - nature.com
Mitochondria are cellular organelles critical for numerous cellular processes and harboring
their own circular mitochondrial DNA (mtDNA). Most mtDNA associated disorders (either …
their own circular mitochondrial DNA (mtDNA). Most mtDNA associated disorders (either …