Search Results - "Castilho, Róger Frigério"
-
1
The Contribution of Nicotinamide Nucleotide Transhydrogenase to Peroxide Detoxification Is Dependent on the Respiratory State and Counterbalanced by Other Sources of NADPH in Liver Mitochondria
Published in The Journal of biological chemistry (16-09-2016)“…The forward reaction of nicotinamide nucleotide transhydrogenase (NNT) reduces NADP+ at the expense of NADH oxidation and H+ movement down the electrochemical…”
Get full text
Journal Article -
2
2‐Methylcitric acid impairs glutamate metabolism and induces permeability transition in brain mitochondria
Published in Journal of neurochemistry (01-04-2016)“…Accumulation of 2‐methylcitric acid (2MCA) is observed in methylmalonic and propionic acidemias, which are clinically characterized by severe neurological…”
Get full text
Journal Article -
3
IGFBP7 Fuels the Glycolytic Metabolism in B-Cell Precursor Acute Lymphoblastic Leukemia by Sustaining Activation of the IGF1R-Akt-GLUT1 Axis
Published in International journal of molecular sciences (02-06-2023)“…Increased glycolytic metabolism plays an important role in B-cell precursor Acute Lymphoblastic Leukemia (BCP-ALL). We previously showed that IGFBP7 exerts…”
Get full text
Journal Article -
4
Sickle-cell Anemia and Latent Diastolic Dysfunction: Echocardiographic Alterations
Published in Arquivos brasileiros de cardiologia (01-04-2015)Get full text
Journal Article -
5
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
Published in Biochemical and biophysical research communications (03-12-2023)“…Aminoacylase 1 (ACY1) deficiency is an inherited metabolic disorder biochemically characterized by high urinary concentrations of aliphatic N-acetylated amino…”
Get full text
Journal Article -
6
Disruption of mitochondrial bioenergetics, calcium retention capacity and cell viability caused by D-2-hydroxyglutaric acid in the heart
Published in Biochimie (01-04-2023)“…Accumulation of D-2-hydroxyglutaric acid (D-2-HG) is the biochemical hallmark of D-2-hydroxyglutaric aciduria type I and, particularly, of D-2-hydroxyglutaric…”
Get full text
Journal Article -
7
Disturbance of bioenergetics and calcium homeostasis provoked by metabolites accumulating in propionic acidemia in heart mitochondria of developing rats
Published in Biochimica et biophysica acta. Molecular basis of disease (01-05-2020)“…Propionic acidemia is caused by lack of propionyl-CoA carboxylase activity. It is biochemically characterized by accumulation of propionic (PA) and…”
Get full text
Journal Article -
8
Disturbance of mitochondrial functions caused by N-acetylglutamate and N-acetylmethionine in brain of adolescent rats: Potential relevance in aminoacylase 1 deficiency
Published in Neurochemistry international (01-12-2023)“…Aminoacylase 1 (ACY1) deficiency is a rare genetic disorder that affects the breakdown of short-chain aliphatic N-acetylated amino acids, leading to the…”
Get full text
Journal Article -
9
Disturbance of mitochondrial functions associated with permeability transition pore opening induced by cis-5-tetradecenoic and myristic acids in liver of adolescent rats
Published in Mitochondrion (01-01-2020)“…•Cis-5-tetradecenoic (Cis-5) and myristic (Myr) acids accumulate in VLCAD deficiency.•Cis-5 and Myr impair respiration in liver mitochondria and permeabilized…”
Get full text
Journal Article -
10
Experimental evidence that maleic acid markedly compromises glutamate oxidation through inhibition of glutamate dehydrogenase and α-ketoglutarate dehydrogenase activities in kidney of developing rats
Published in Molecular and cellular biochemistry (01-08-2019)“…Maleic acid (MA), which has been reported to be highly excreted in propionic acidemia (PAcidemia), was demonstrated to cause nephropathy by bioenergetics…”
Get full text
Journal Article -
11
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
Published in Biochimica et biophysica acta (01-06-2015)“…Patients with long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) deficiency commonly present liver dysfunction whose pathogenesis is unknown. We studied the…”
Get full text
Journal Article -
12
Metabolite accumulation in VLCAD deficiency markedly disrupts mitochondrial bioenergetics and Ca2+ homeostasis in the heart
Published in The FEBS journal (01-04-2018)“…We studied the effects of the major long‐chain fatty acids accumulating in very long‐chain acyl‐CoA dehydrogenase (VLCAD) deficiency, namely…”
Get full text
Journal Article -
13
Toxic effects of phytol and retinol on human glioblastoma cells are associated with modulation of cholesterol and fatty acid biosynthetic pathways
Published in Journal of neuro-oncology (01-02-2018)“…Glioblastoma (GBM) is the most common primary brain tumor. Genetic mutations may reprogram the metabolism of neoplastic cells. Particularly, alterations in…”
Get full text
Journal Article -
14
Sulfasalazine intensifies temozolomide cytotoxicity in human glioblastoma cells
Published in Molecular and cellular biochemistry (01-07-2016)“…Temozolomide (TMZ) is an alkylating agent used to treat glioblastoma. This tumor type synthesizes the antioxidant glutathione through system X c − , which is…”
Get full text
Journal Article -
15
Characteristics of sulfasalazine-induced cytotoxicity in C6 rat glioma cells
Published in Neuroscience letters (18-01-2017)“…Glioblastoma is the most aggressive primary brain tumor. Surgical resection, radiotherapy and temozolomide (TMZ), an alkylating agent, is the standard of care…”
Get full text
Journal Article -
16
Ethylmalonic Acid Induces Permeability Transition in Isolated Brain Mitochondria
Published in Neurotoxicity research (01-08-2014)“…Predominant accumulation of ethylmalonic acid (EMA) in tissues and biological fluids is a characteristic of patients affected by short chain acyl-CoA…”
Get full text
Journal Article -
17
pCramoll and rCramoll lectins induce cell death in human prostate adenocarcinoma (PC-3) cells by impairment of mitochondrial homeostasis
Published in Toxicology in vitro (01-09-2017)“…Lectins from Cratylia mollis seed have shown potential in vivo antitumor actions, however the mechanism have not yet been addressed. Here we evaluated the…”
Get full text
Journal Article -
18
Mitochondrial NAD(P) + Transhydrogenase: From Molecular Features to Physiology and Disease
Published in Antioxidants & redox signaling (01-05-2022)“…Proton-translocating NAD(P) transhydrogenase, also known as nicotinamide nucleotide transhydrogenase (NNT), catalyzes a reversible reaction coupling the…”
Get more information
Journal Article -
19
Ethylmalonic acid impairs brain mitochondrial succinate and malate transport
Published in Molecular genetics and metabolism (01-01-2012)“…Tissue accumulation and high urinary excretion of ethylmalonic acid (EMA) occur in ethylmalonic encephalopathy (EE) and short chain acyl-CoA dehydrogenase…”
Get full text
Journal Article -
20
Impairment of mitochondrial bioenergetics and permeability transition induction caused by major long-chain fatty acids accumulating in VLCAD deficiency in skeletal muscle as potential pathomechanisms of myopathy
Published in Toxicology in vitro (01-02-2020)“…cis-5-Tetradecenoic (cis-5) and myristic (Myr) acids predominantly accumulate in patients affected by very long-chain acyl-CoA dehydrogenase (VLCAD)…”
Get full text
Journal Article