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A humán dihidrolipoamid- dehidrogenáz deficiencia molekuláris patomechanizmusa

Szabó, Eszter; Ambrus, Attila (2017)
A multipronged approach unravels unprecedented protein-protein interactions in the human 2-oxoglutarate dehydrogenase multienzyme complex

Zhou Jieyu; Yang Luying; Ozohanics Oliver; Zhang Xu; Wang Junjie; Ambrus, Attila; Arjunan Palaniappa; Brukh Roman; Nemeria Natalia S; Furey William; Jordan Frank (2018)

The human 2-oxoglutaric acid dehydrogenase complex (hOGDHc) plays a pivotal role in the tricarboxylic acid (TCA) cycle, and its diminished activity is associated with neurodegenerative diseases. The hOGDHc comprises three ...
An updated view on the molecular pathomechanisms of human dihydrolipoamide dehydrogenase deficiency in light of novel crystallographic evidence

Ambrus, Attila (2019)

Dihydrolipoamide dehydrogenase (LADH, E3) deficiency is a rare (autosomal, recessive) genetic disorder generally presenting with an onset in the neonatal age and early death; the highest carrier rate has been found among ...
Crystal structures of the disease-causing D444V mutant and the relevant wild type human dihydrolipoamide dehydrogenase

Szabó, Eszter; Mizsei, Réka; Wilk, P; Zambo, Z; Töröcsik, Beáta; Weiss MS; Ádám, Veronika; Ambrus, Attila (2018)

We report the crystal structures of the human (dihydro)lipoamide dehydrogenase (hLADH, hE3) and its disease-causing homodimer interface mutant D444V-hE3 at 2.27 and 1.84 Å resolution, respectively. The wild type structure ...
Exclusive neuronal detection of KGDHC-specific subunits in the adult human brain cortex despite pancellular protein lysine succinylation

Dobolyi, Árpád; Bagó, Attila György; Palkovits, Miklós; Nemeria, NS; Jordan, F; Dóczi, Judit; Ambrus, Attila; Ádám, Veronika; Chinopoulos, Christos (2020)
Formation of reactive oxygen species by human and bacterial pyruvate and 2- oxoglutarate dehydrogenase multienzyme complexes reconstituted from recombinant components

Ambrus, Attila; Nemeria, Natália S; Törőcsik, Beáta; Tretter, László; Nilsson, Mattias; Frank, Jordan; Ádám, Veronika (2015)
Human dihydrolipoamide dehydrogenase (E3) deficiency: Novel insights into the structural basis and molecular pathomechanism

Ambrus, Attila; Ádám-Vizi, Veronika (2018)

This review summarizes our present view on the molecular pathogenesis of human (h) E3-deficiency caused by a variety of genetic alterations with a special emphasis on the moonlighting biochemical phenomena related to the ...
Measurement of ROS homeostasis in isolated mitochondria

Tretter, László; Ambrus, Attila (2014)
Membrane potential and delta pH dependency of reverse electron transport-associated hydrogen peroxide production in brain and heart mitochondria

Komlódi, Tímea; Geibl FF; Sassani M; Ambrus, Attila; Tretter, László (2018)

Succinate-driven reverse electron transport (RET) is one of the main sources of mitochondrial reactive oxygen species (mtROS) in ischemia-reperfusion injury. RET is dependent on mitochondrial membrane potential (Deltapsim) ...
Structural alterations by five disease-causing mutations in the low-pH conformation of human dihydrolipoamide dehydrogenase (hLADH) analyzed by molecular dynamics – implications in functional loss and modulation of reactive oxygen species generation by pathogenic hLADH forms

Ambrus, Attila; Mizsei, Réka; Ádám, Veronika (2015)
Structural alterations induced by ten disease-causing mutations of human dihydrolipoamide dehydrogenase analyzed by hydrogen/deuterium-exchange mass spectrometry: Implications for the structural basis of E3 deficiency

Ambrus, Attila; Wang Junjie; Mizsei, Réka; Zámbó, Zsófia; Törőcsik, Beáta; Jordan Frank; Ádám-Vizi, Veronika (2016)

Pathogenic amino acid substitutions of the common E3 component (hE3) of the human alpha-ketoglutarate dehydrogenase and the pyruvate dehydrogenase complexes lead to severe metabolic diseases (E3 deficiency), which usually ...
The Mitochondrial Targets of Neuroprotective Drug Vinpocetine on Primary Neuron Cultures, Brain Capillary Endothelial Cells, Synaptosomes, and Brain Mitochondria

Sváb, Gergely; Dóczi, Judit; Gerencser, Akos A; Ambrus, Attila; Gallyas, Ferenc; Sümegi, Balázs; Tretter, László (2019)

Vinpocetine is considered as neuroprotectant drug and used for treatment of brain ischemia and cognitive deficiencies for decades. A number of enzymes, channels and receptors can bind vinpocetine, however the mechanisms ...
TP53 mutation hits energy metabolism and increases glycolysis in breast cancer

Harami-Papp H; Pongor, Lőrinc; Munkacsy G; Horváth, Gergő; Nagy, Ádám Miklós; Ambrus, Attila; Hauser, Péter; Szabó, András; Tretter, László; Győrffy, Balázs (2016)

Promising new hallmarks of cancer is alteration of energy metabolism that involves molecular mechanisms shifting cancer cells to aerobe glycolysis. Our goal was to evaluate the correlation between mutation in the commonly ...
Triethylene glycol dimethacrylate impairs bioenergetic functions and induces oxidative stress in mitochondria via inhibiting respiratory Complex I

Mikulás, Krisztina; Hermann, Péter; Gera, István; Komlódi, Tímea; Horváth, Gergő; Ambrus, Attila; Tretter, László (2018)

OBJECTIVES: Earlier studies demonstrated that dental resin monomers lower cellular viability and provoke oxidative stress. Reactive oxygen species (ROS) formation has a key role in triethylene glycol dimethacrylate (TEGDMA) ...

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