Detalhe da pesquisa
1.
Effect of genistein and coenzyme Q10 in oxidative damage and mitochondrial membrane potential in an attenuated type II mucopolysaccharidosis cellular model.
Cell Biochem Funct
; 42(2): e3932, 2024 Mar.
Artigo
em Inglês
| MEDLINE | ID: mdl-38332678
2.
Evaluation of biochemical profile and oxidative damage to lipids and proteins in patients with lysosomal acid lipase deficiency.
Biochem Cell Biol
; 101(4): 294-302, 2023 08 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-37042460
3.
Inflammatory process and oxidative/nitrative stress: in vivo study in mucopolysaccharidosis type IV A patients under long-term enzyme replacement therapy.
Arch Biochem Biophys
; 737: 109541, 2023 03 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-36754222
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.
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
6.
Evaluation of oxidative stress and mitochondrial function in a type II mucopolysaccharidosis cellular model: in vitro effects of genistein and coenzyme Q10.
Metab Brain Dis
; 38(2): 519-529, 2023 02.
Artigo
em Inglês
| MEDLINE | ID: mdl-36029429
7.
Evidence that Oxidative Disbalance and Mitochondrial Dysfunction are Involved in the Pathophysiology of Fatty Acid Oxidation Disorders.
Cell Mol Neurobiol
; 42(3): 521-532, 2022 Apr.
Artigo
em Inglês
| MEDLINE | ID: mdl-32876899
8.
Hyperammonemia in Inherited Metabolic Diseases.
Cell Mol Neurobiol
; 42(8): 2593-2610, 2022 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-34665389
9.
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
10.
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
11.
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
12.
Preliminary results of PBA-loaded nanoparticles development and the effect on oxidative stress and neuroinflammation in rats submitted to a chemically induced chronic model of MSUD.
Metab Brain Dis
; 36(5): 1015-1027, 2021 06.
Artigo
em Inglês
| MEDLINE | ID: mdl-33620579
13.
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
14.
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
15.
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
16.
Information and Diagnosis Networks - tools to improve diagnosis and treatment for patients with rare genetic diseases.
Genet Mol Biol
; 42(1 suppl 1): 155-164, 2019.
Artigo
em Inglês
| MEDLINE | ID: mdl-31188934
17.
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.
Artigo
em Inglês
| MEDLINE | ID: mdl-29923625
18.
Oxidative damage in glutaric aciduria type I patients and the protective effects of l-carnitine treatment.
J Cell Biochem
; 119(12): 10021-10032, 2018 12.
Artigo
em Inglês
| MEDLINE | ID: mdl-30129250
19.
Inflammatory profile in X-linked adrenoleukodystrophy patients: Understanding disease progression.
J Cell Biochem
; 119(1): 1223-1233, 2018 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-28722826
20.
Oxidative and nitrative stress and pro-inflammatory cytokines in Mucopolysaccharidosis type II patients: effect of long-term enzyme replacement therapy and relation with glycosaminoglycan accumulation.
Biochim Biophys Acta
; 1862(9): 1608-16, 2016 09.
Artigo
em Inglês
| MEDLINE | ID: mdl-27251652