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1.
Disturbances in mitochondrial bioenergetics and control quality and unbalanced redox homeostasis in the liver of a mouse model of mucopolysaccharidosis type II.
Mol Cell Biochem
; 2024 Mar 18.
Article
in English
| MEDLINE | ID: mdl-38498105
2.
N-Acetylglutamate and N-acetylmethionine compromise mitochondrial bioenergetics homeostasis and glutamate oxidation in brain of developing rats: Potential implications for the pathogenesis of ACY1 deficiency.
Biochem Biophys Res Commun
; 684: 149123, 2023 12 03.
Article
in English
| MEDLINE | ID: mdl-37871522
3.
Disruption of mitochondrial functions involving mitochondrial permeability transition pore opening caused by maleic acid in rat kidney.
J Bioenerg Biomembr
; 54(4): 203-213, 2022 Aug.
Article
in English
| MEDLINE | ID: mdl-35902433
4.
S-adenosylmethionine induces mitochondrial dysfunction, permeability transition pore opening and redox imbalance in subcellular preparations of rat liver.
J Bioenerg Biomembr
; 53(5): 525-539, 2021 10.
Article
in English
| MEDLINE | ID: mdl-34347214
5.
Disruption of mitochondrial functions and oxidative stress contribute to neurologic dysfunction in organic acidurias.
Arch Biochem Biophys
; 696: 108646, 2020 12 15.
Article
in English
| MEDLINE | ID: mdl-33098870
6.
Experimental evidence that maleic acid markedly compromises glutamate oxidation through inhibition of glutamate dehydrogenase and α-ketoglutarate dehydrogenase activities in kidney of developing rats.
Mol Cell Biochem
; 458(1-2): 99-112, 2019 Aug.
Article
in English
| MEDLINE | ID: mdl-31032535
7.
High vulnerability of the heart and liver to 3-hydroxypalmitic acid-induced disruption of mitochondrial functions in intact cell systems.
J Cell Biochem
; 119(9): 7678-7686, 2018 09.
Article
in English
| MEDLINE | ID: mdl-29923625
8.
cis-4-Decenoic and decanoic acids impair mitochondrial energy, redox and Ca(2+) homeostasis and induce mitochondrial permeability transition pore opening in rat brain and liver: Possible implications for the pathogenesis of MCAD deficiency.
Biochim Biophys Acta
; 1857(9): 1363-1372, 2016 09.
Article
in English
| MEDLINE | ID: mdl-27240720
9.
Bioenergetics dysfunction, mitochondrial permeability transition pore opening and lipid peroxidation induced by hydrogen sulfide as relevant pathomechanisms underlying the neurological dysfunction characteristic of ethylmalonic encephalopathy.
Biochim Biophys Acta Mol Basis Dis
; 1863(9): 2192-2201, 2017 09.
Article
in English
| MEDLINE | ID: mdl-28624490
10.
Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency.
Biochim Biophys Acta
; 1847(6-7): 620-8, 2015.
Article
in English
| MEDLINE | ID: mdl-25868874
11.
Disturbance of energy and redox homeostasis and reduction of Na+,K+-ATPase activity provoked by in vivo intracerebral administration of ethylmalonic acid to young rats.
Biochim Biophys Acta
; 1852(5): 759-67, 2015 May.
Article
in English
| MEDLINE | ID: mdl-25583115
12.
2-Methylcitric acid impairs glutamate metabolism and induces permeability transition in brain mitochondria.
J Neurochem
; 137(1): 62-75, 2016 Apr.
Article
in English
| MEDLINE | ID: mdl-26800654
13.
Ornithine and Homocitrulline Impair Mitochondrial Function, Decrease Antioxidant Defenses and Induce Cell Death in Menadione-Stressed Rat Cortical Astrocytes: Potential Mechanisms of Neurological Dysfunction in HHH Syndrome.
Neurochem Res
; 41(9): 2190-8, 2016 Sep.
Article
in English
| MEDLINE | ID: mdl-27161368
14.
Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: a possible role of mPTP opening as a pathomechanism in these disorders?
Biochim Biophys Acta
; 1842(9): 1658-67, 2014 Sep.
Article
in English
| MEDLINE | ID: mdl-24946182
15.
Disruption of brain redox homeostasis in glutaryl-CoA dehydrogenase deficient mice treated with high dietary lysine supplementation.
Mol Genet Metab
; 108(1): 30-9, 2013 Jan.
Article
in English
| MEDLINE | ID: mdl-23218171
16.
Disruption of mitochondrial homeostasis by phytanic acid in cerebellum of young rats.
Cerebellum
; 12(3): 362-9, 2013 Jun.
Article
in English
| MEDLINE | ID: mdl-23081695
17.
L-2-Hydroxyglutaric Acid Administration to Neonatal Rats Elicits Marked Neurochemical Alterations and Long-Term Neurobehavioral Disabilities Mediated by Oxidative Stress.
Neurotox Res
; 41(2): 119-140, 2023 Apr.
Article
in English
| MEDLINE | ID: mdl-36580261
18.
Disruption of mitochondrial bioenergetics, calcium retention capacity and cell viability caused by D-2-hydroxyglutaric acid in the heart.
Biochimie
; 207: 153-164, 2023 Apr.
Article
in English
| MEDLINE | ID: mdl-36372308
19.
Disruption of mitochondrial bioenergetics and calcium homeostasis by phytanic acid in the heart: Potential relevance for the cardiomyopathy in Refsum disease.
Biochim Biophys Acta Bioenerg
; 1864(2): 148961, 2023 04 01.
Article
in English
| MEDLINE | ID: mdl-36812958
20.
Disturbance of mitochondrial functions caused by N-acetylglutamate and N-acetylmethionine in brain of adolescent rats: Potential relevance in aminoacylase 1 deficiency.
Neurochem Int
; 171: 105631, 2023 Dec.
Article
in English
| MEDLINE | ID: mdl-37852579