(Q39588663)

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3-Hydroxy-3-methylglutaric and 3-methylglutaric acids impair redox status and energy production and transfer in rat heart: relevance for the pathophysiology of cardiac dysfunction in 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency.

scientific article published on 18 July 2016

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19 March 2020

3-Hydroxy-3-methylglutaric and 3-methylglutaric acids impair redox status and energy production and transfer in rat heart: relevance for the pathophysiology of cardiac dysfunction in 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (English)

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19 March 2020

pathophysiology

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Bianca Seminotti

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19 March 2020

César Augusto João Ribeiro

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19 March 2020

Belisa Parmeggiani

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19 March 2020

Guilhian Leipnitz

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19 March 2020

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Moacir Wajner

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19 March 2020

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Mateus Struecker da Rosa

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19 March 2020

Mateus Grings

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23 August 2016

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19 March 2020

Free Radical Research

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19 March 2020

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19 March 2020

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19 March 2020

997-1010

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19 March 2020

Differential HMG-CoA lyase expression in human tissues provides clues about 3-hydroxy-3-methylglutaric aciduria

1 reference

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21 January 2018

Evidence that 3-hydroxy-3-methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex.

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21 January 2018

Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats.

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In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: a potential pathophysiological mechanism of striatal damage in this diso

1 reference

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21 January 2018

Redox homeostasis is compromised in vivo by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in rat cerebral cortex and liver.

1 reference

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21 January 2018

Disturbance of brain energy and redox homeostasis provoked by sulfite and thiosulfate: potential pathomechanisms involved in the neuropathology of sulfite oxidase deficiency.

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α-Lipoic acid protected cardiomyoblasts from the injury induced by sodium nitroprusside through ROS-mediated Akt/Gsk-3β activation

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In vitro stimulation of oxidative stress by hypoxanthine in blood of rats: prevention by vitamins e plus C and allopurinol

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Inhibition of cytochrome c oxidase activity in rat cerebral cortex and human skeletal muscle by D-2-hydroxyglutaric acid in vitro.

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21 January 2018

Inhibition of creatine kinase activity from rat cerebral cortex by D-2-hydroxyglutaric acid in vitro.

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Catalase in vitro

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21 January 2018

3-Hydroxy-3-methylglutaric aciduria in an Italian patient is caused by a new nonsense mutation in the HMGCL gene.

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21 January 2018

3-Hydroxy-3-methylglutaryl coenzyme a lyase deficiency with reversible white matter changes after treatment

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Differential effects of peroxynitrite on human mitochondrial creatine kinase isoenzymes. Inactivation, octamer destabilization, and identification of involved residues

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21 January 2018

Mitochondrial dysfunction in heart failure

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Creatine kinase and ATP synthase reaction rates in human frontal lobe measured by ³¹P magnetization transfer spectroscopy at 4T.

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Hydrogen peroxide targets the cysteine at the active site and irreversibly inactivates creatine kinase.

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An unusually low pK(a) for Cys282 in the active site of human muscle creatine kinase

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Rapid and irreversible inhibition of creatine kinase by peroxynitrite

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Oligomeric state and membrane binding behaviour of creatine kinase isoenzymes: implications for cellular function and mitochondrial structure.

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Protein carbonyl groups as biomarkers of oxidative stress.

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H2O2 and tumor necrosis factor-alpha induce differential binding of the redox-responsive transcription factors AP-1 and NF-kappaB to the interleukin-8 promoter in endothelial and epithelial cells

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Oxidative stress and neurodegeneration: where are we now?

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Inactivation of human Cu,Zn superoxide dismutase by peroxynitrite and formation of histidinyl radical.

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Inactivation of catalase and superoxide dismutase by singlet oxygen derived from photoactivated dye.

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21 January 2018

Oleanolic acid: a novel cardioprotective agent that blunts hyperglycemia-induced contractile dysfunction.

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Partial inhibition of the ubiquitin-proteasome system ameliorates cardiac dysfunction following ischemia-reperfusion in the presence of high glucose

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Vitamin D treatment protects against and reverses oxidative stress induced muscle proteolysis

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Impaired enzymatic defensive activity, mitochondrial dysfunction and proteasome activation are involved in RTT cell oxidative damage

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Regulation of antioxidant enzymes: a significant role for melatonin

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Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency

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Protective effects of melatonin in reducing oxidative stress and in preserving the fluidity of biological membranes: a review

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10.1080/10715762.2016.1214952

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Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27430492%20AND%20SRC:MED&resulttype=core&format=json

19 March 2020

PubMed publication ID

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Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27430492%20AND%20SRC:MED&resulttype=core&format=json

19 March 2020

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