MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options

AW El-Hattab, AM Adesina, J Jones… - Molecular genetics and …, 2015 - Elsevier
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)
syndrome is one of the most frequent maternally inherited mitochondrial disorders. MELAS …

Therapies for mitochondrial diseases and current clinical trials

AW El-Hattab, AM Zarante, M Almannai… - Molecular genetics and …, 2017 - Elsevier
Mitochondrial diseases are a clinically and genetically heterogeneous group of disorders
that result from dysfunction of the mitochondrial oxidative phosphorylation due to molecular …

Unveiling the human nitroproteome: Protein tyrosine nitration in cell signaling and cancer

I Griswold-Prenner, AK Kashyap, S Mazhar… - Journal of Biological …, 2023 - ASBMB
Covalent amino acid modification significantly expands protein functional capability in
regulating biological processes. Tyrosine residues can undergo phosphorylation, sulfation …

Restoration of impaired nitric oxide production in MELAS syndrome with citrulline and arginine supplementation

AW El-Hattab, JW Hsu, LT Emrick, LJC Wong… - Molecular genetics and …, 2012 - Elsevier
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)
syndrome is one of the most common mitochondrial disorders. Although the pathogenesis of …

Inhibition of peroxynitrite-induced mitophagy activation attenuates cerebral ischemia-reperfusion injury

J Feng, X Chen, B Guan, C Li, J Qiu, J Shen - Molecular neurobiology, 2018 - Springer
Activated autophagy/mitophagy has been intensively observed in ischemic brain, but its
roles remain controversial. Peroxynitrite (ONOO−), as a representative of reactive nitrogen …

Oxidative stress and mitochondrial dysfunction across broad‐ranging pathologies: toward mitochondria‐targeted clinical strategies

G Pagano, A Aiello Talamanca… - Oxidative medicine …, 2014 - Wiley Online Library
Beyond the disorders recognized as mitochondrial diseases, abnormalities in function
and/or ultrastructure of mitochondria have been reported in several unrelated pathologies …

Naringin attenuates cerebral ischemia-reperfusion injury through inhibiting peroxynitrite-mediated mitophagy activation

J Feng, X Chen, S Lu, W Li, D Yang, W Su… - Molecular …, 2018 - Springer
Excessive autophagy/mitophagy plays important roles during cerebral ischemia-reperfusion
(I/R) injury. Peroxynitrite (ONOO−), a representative reactive nitrogen species, mediates …

Therapeutic approaches to treat mitochondrial diseases:“one-size-fits-all” and “precision medicine” strategies

E Bottani, C Lamperti, A Prigione, V Tiranti, N Persico… - Pharmaceutics, 2020 - mdpi.com
Primary mitochondrial diseases (PMD) refer to a group of severe, often inherited genetic
conditions due to mutations in the mitochondrial genome or in the nuclear genes encoding …

Desmin reorganization by stimuli inducing oxidative stress and electrophiles: role of its single cysteine residue

D Moneo-Corcuera, Á Viedma-Poyatos, K Stamatakis… - Antioxidants, 2023 - mdpi.com
The type III intermediate filament proteins vimentin and GFAP are modulated by oxidants
and electrophiles, mainly through perturbation of their single cysteine residues. Desmin, the …

Impaired nitric oxide production in children with MELAS syndrome and the effect of arginine and citrulline supplementation

AW El-Hattab, LT Emrick, JW Hsu… - Molecular genetics and …, 2016 - Elsevier
Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)
syndrome is one of the most frequent maternally inherited mitochondrial disorders. The …