Detalhe da pesquisa
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.
Artigo
em Inglês
| 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.
Artigo
em Inglês
| MEDLINE | ID: mdl-37871522
3.
Evaluation of oxidative damage to biomolecules and inflammation in patients with urea cycle disorders.
Arch Biochem Biophys
; 736: 109526, 2023 03 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-36702451
4.
Increased peripheral of brain-derived neurotrophic factor levels in phenylketonuric patients treated with l-carnitine.
Arch Biochem Biophys
; 749: 109792, 2023 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-37863349
5.
Sulfite Impairs Bioenergetics and Redox Status in Neonatal Rat Brain: Insights into the Early Neuropathophysiology of Isolated Sulfite Oxidase and Molybdenum Cofactor Deficiencies.
Cell Mol Neurobiol
; 43(6): 2895-2907, 2023 Aug.
Artigo
em Inglês
| MEDLINE | ID: mdl-36862242
6.
Increased cytokine levels induced by high phenylalanine concentrations in late diagnosis PKU patients compared to early diagnosis: Anti-inflammatory effect of L-carnitine.
Cell Biochem Funct
; 41(4): 490-500, 2023 Jun.
Artigo
em Inglês
| MEDLINE | ID: mdl-37170672
7.
Early effects of LPS-induced neuroinflammation on the rat hippocampal glycolytic pathway.
J Neuroinflammation
; 19(1): 255, 2022 Oct 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-36221097
8.
Increased susceptibility to quinolinic acid-induced seizures and long-term changes in brain oscillations in an animal model of glutaric acidemia type I.
J Neurosci Res
; 100(4): 992-1007, 2022 04.
Artigo
em Inglês
| MEDLINE | ID: mdl-34713466
9.
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.
Artigo
em Inglês
| MEDLINE | ID: mdl-35902433
10.
Mitochondrial Dysfunction and Redox Homeostasis Impairment as Pathomechanisms of Brain Damage in Ethylmalonic Encephalopathy: Insights from Animal and Human Studies.
Cell Mol Neurobiol
; 42(3): 565-575, 2022 Apr.
Artigo
em Inglês
| MEDLINE | ID: mdl-33034777
11.
Methylmalonic acid induces inflammatory response and redox homeostasis disruption in C6 astroglial cells: potential glioprotective roles of melatonin and resveratrol.
Amino Acids
; 54(11): 1505-1517, 2022 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-35927507
12.
Ethylmalonic acid impairs bioenergetics by disturbing succinate and glutamate oxidation and induces mitochondrial permeability transition pore opening in rat cerebellum.
J Neurochem
; 158(2): 262-281, 2021 07.
Artigo
em Inglês
| MEDLINE | ID: mdl-33837559
13.
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.
Artigo
em Inglês
| MEDLINE | ID: mdl-34347214
14.
Protective effects of L-carnitine on behavioral alterations and neuroinflammation in striatum of glutaryl-COA dehydrogenase deficient mice.
Arch Biochem Biophys
; 709: 108970, 2021 09 30.
Artigo
em Inglês
| MEDLINE | ID: mdl-34181873
15.
The mitochondrial-targeted reactive species scavenger JP4-039 prevents sulfite-induced alterations in antioxidant defenses, energy transfer, and cell death signaling in striatum of rats.
J Inherit Metab Dis
; 44(2): 481-491, 2021 03.
Artigo
em Inglês
| MEDLINE | ID: mdl-32882059
16.
Clinical, biochemical and molecular findings of 24 Brazilian patients with glutaric acidemia type 1: 4 novel mutations in the GCDH gene.
Metab Brain Dis
; 36(2): 205-212, 2021 02.
Artigo
em Inglês
| MEDLINE | ID: mdl-33064266
17.
L-carnitine protects DNA oxidative damage induced by phenylalanine and its keto acid derivatives in neural cells: a possible pathomechanism and adjuvant therapy for brain injury in phenylketonuria.
Metab Brain Dis
; 36(7): 1957-1968, 2021 10.
Artigo
em Inglês
| MEDLINE | ID: mdl-34216350
18.
Disruption of mitochondrial functions and oxidative stress contribute to neurologic dysfunction in organic acidurias.
Arch Biochem Biophys
; 696: 108646, 2020 12 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-33098870
19.
Oxidative damage in mitochondrial fatty acids oxidation disorders patients and the in vitro effect of l-carnitine on DNA damage induced by the accumulated metabolites.
Arch Biochem Biophys
; 679: 108206, 2020 01 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-31760122
20.
Prevention by L-carnitine of DNA damage induced by 3-hydroxy-3-methylglutaric and 3-methylglutaric acids and experimental evidence of lipid and DNA damage in patients with 3-hydroxy-3-methylglutaric aciduria.
Arch Biochem Biophys
; 668: 16-22, 2019 06 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-31047871