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Electron transfer flavoprotein and its role in mitochondrial energy metabolism in health and disease

Gene, 2021-04, Vol.776, p.145407-145407, Article 145407

2021

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Functional Recovery of a GCDH Variant Associated to Severe Deflavinylation-Molecular Insights into Potential Beneficial Effects of Riboflavin Supplementation in Glutaric Aciduria-Type I Patients

International journal of molecular sciences, 2020-10, Vol.21 (19), p.7063

2020

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Therapeutic Approaches Using Riboflavin in Mitochondrial Energy Metabolism Disorders

Current drug targets, 2016-01, Vol.17 (13), p.1527

2016

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Conformational analysis of the riboflavin-responsive ETF:QO-p.Pro456Leu variant associated with mild multiple acyl-CoA dehydrogenase deficiency

Biochimica et biophysica acta. Proteins and proteomics, 2020-06, Vol.1868 (6), p.140393-140393, Article 140393

2020

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Emerging roles for riboflavin in functional rescue of mitochondrial β-oxidation flavoenzymes

Current medicinal chemistry, 2010-11, Vol.17 (32), p.3842

2010

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Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance

Biochimica et biophysica acta. Proteins and proteomics, 2020-01, Vol.1868 (1), p.140269-140269, Article 140269

2020

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Deglutarylation of glutaryl-CoA dehydrogenase by deacylating enzyme SIRT5 promotes lysine oxidation in mice

The Journal of biological chemistry, 2022-04, Vol.298 (4), p.101723-101723, Article 101723

2022

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Biophysical and Spectroscopic Methods for Monitoring Protein Misfolding and Amyloid Aggregation

Methods in molecular biology (Clifton, N.J.), 2019, Vol.1873, p.3

2019

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Cofactors and metabolites as protein folding helpers in metabolic diseases

Current topics in medicinal chemistry, 2012-11, Vol.12 (22), p.2546

2012

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Ethylmalonic encephalopathy ETHE1 R163W/R163Q mutations alter protein stability and redox properties of the iron centre

PloS one, 2014-09, Vol.9 (9), p.e107157-e107157

2014

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Cofactors and metabolites as potential stabilizers of mitochondrial acyl-CoA dehydrogenases

Biochimica et biophysica acta, 2011-12, Vol.1812 (12), p.1658-1663

2011

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Mutational hotspots in electron transfer flavoprotein underlie defective folding and function in multiple acyl-CoA dehydrogenase deficiency

Biochimica et biophysica acta, 2010-11, Vol.1802 (11), p.1070-1077

2010

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A polymorphic position in electron transfer flavoprotein modulates kinetic stability as evidenced by thermal stress

FEBS letters, 2011-02, Vol.585 (3), p.505-510

2011

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Mutations at the flavin binding site of ETF:QO yield a MADD-like severe phenotype in Drosophila

Biochimica et biophysica acta, 2012-08, Vol.1822 (8), p.1284-1292

2012

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Probing the mechanism of rubredoxin thermal unfolding in the absence of salt bridges by temperature jump experiments

Biochemical and biophysical research communications, 2005-08, Vol.333 (3), p.839-844

2005

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Role of Flavinylation in a Mild Variant of Multiple Acyl-CoA Dehydrogenation Deficiency: A MOLECULAR RATIONALE FOR THE EFFECTS OF RIBOFLAVIN SUPPLEMENTATION

The Journal of biological chemistry, 2009-02, Vol.284 (7), p.4222-4229

2009

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Role of Flavinylation in a Mild Variant of Multiple Acyl-CoA Dehydrogenation Deficiency

The Journal of biological chemistry, 2009-02, Vol.284 (7), p.4222-4229

2009

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Combined spectroscopic and calorimetric characterisation of rubredoxin reversible thermal transition

Journal of biological inorganic chemistry, 2006-01, Vol.11 (1), p.73-81

2006

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P-328 - Implication of post-translational modifications on ETF, a structural and functional approach

Free radical biology & medicine, 2018-05, Vol.120, p.S144-S144

2018

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Implication of post-translational modifications on ETF, a structural and functional approach

Free radical biology & medicine, 2018-05, Vol.120, p.S144

2018

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P-327 - Structural and functional effects of clinical missense mutations on glutaryl-CoA dehydrogenase

Free radical biology & medicine, 2018-05, Vol.120, p.S144-S144

2018

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Structural and functional effects of clinical missense mutations on glutaryl-CoA dehydrogenase

Free radical biology & medicine, 2018-05, Vol.120, p.S144

2018

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Chapter 12 - Riboflavin (vitamin B2) and mitochondrial energy

Molecular Nutrition, 2020, p.225-244

2020

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João Guimarães Rosa : Mémoire et imaginaire du sertão-monde

2012

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Electron transfer flavoprotein and its role in mitochondrial energy metabolism in health and disease

Functional Recovery of a GCDH Variant Associated to Severe Deflavinylation-Molecular Insights into Potential Beneficial Effects of Riboflavin Supplementation in Glutaric Aciduria-Type I Patients

Therapeutic Approaches Using Riboflavin in Mitochondrial Energy Metabolism Disorders

Conformational analysis of the riboflavin-responsive ETF:QO-p.Pro456Leu variant associated with mild multiple acyl-CoA dehydrogenase deficiency

Emerging roles for riboflavin in functional rescue of mitochondrial β-oxidation flavoenzymes

Potential complementation effects of two disease-associated mutations in tetrameric glutaryl-CoA dehydrogenase is due to inter subunit stability-activity counterbalance

Deglutarylation of glutaryl-CoA dehydrogenase by deacylating enzyme SIRT5 promotes lysine oxidation in mice

Biophysical and Spectroscopic Methods for Monitoring Protein Misfolding and Amyloid Aggregation

Cofactors and metabolites as protein folding helpers in metabolic diseases

Ethylmalonic encephalopathy ETHE1 R163W/R163Q mutations alter protein stability and redox properties of the iron centre

Cofactors and metabolites as potential stabilizers of mitochondrial acyl-CoA dehydrogenases

Mutational hotspots in electron transfer flavoprotein underlie defective folding and function in multiple acyl-CoA dehydrogenase deficiency

A polymorphic position in electron transfer flavoprotein modulates kinetic stability as evidenced by thermal stress

Mutations at the flavin binding site of ETF:QO yield a MADD-like severe phenotype in Drosophila

Probing the mechanism of rubredoxin thermal unfolding in the absence of salt bridges by temperature jump experiments

Role of Flavinylation in a Mild Variant of Multiple Acyl-CoA Dehydrogenation Deficiency: A MOLECULAR RATIONALE FOR THE EFFECTS OF RIBOFLAVIN SUPPLEMENTATION

Role of Flavinylation in a Mild Variant of Multiple Acyl-CoA Dehydrogenation Deficiency

Combined spectroscopic and calorimetric characterisation of rubredoxin reversible thermal transition

P-328 - Implication of post-translational modifications on ETF, a structural and functional approach

Implication of post-translational modifications on ETF, a structural and functional approach

P-327 - Structural and functional effects of clinical missense mutations on glutaryl-CoA dehydrogenase

Structural and functional effects of clinical missense mutations on glutaryl-CoA dehydrogenase

Chapter 12 - Riboflavin (vitamin B2) and mitochondrial energy

João Guimarães Rosa : Mémoire et imaginaire du sertão-monde

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