Bárbara J Henriques

发表

Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction.

Marisa Sousa, M. Amaral, C. Farinha, 2013, Chemistry & biology.

Deglutarylation of GCDH by SIRT5 controls lysine metabolism in mice

O. Ilkayeva, C. Gomes, P. Grimsrud, 2020, bioRxiv.

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

P. Bross, C. Gomes, Bárbara J Henriques, 2010, Current medicinal chemistry.

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

C. Gomes, L. M. Saraiva, Bárbara J Henriques, 2005, Biochemical and biophysical research communications.

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

P. Bross, C. Gomes, Bárbara J Henriques, 2021, Gene.

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

C. Gomes, Bárbara J Henriques, J. V. Ribeiro, 2020, International journal of molecular sciences.

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

P. Bross, C. Gomes, Bárbara J Henriques, 2019, Biochimica et biophysica acta. Proteins and proteomics.

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

P. Bross, C. Gomes, Bárbara J Henriques, 2010, Biochimica et biophysica acta.

Defective protein folding and function in metabolic disorders: studies on the mitochondrial flavoenzyme ETF

Bárbara J Henriques, 2010 .

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

C. Gomes, Bárbara J Henriques, Tânia G. Lucas, 2020, Biochimica et biophysica acta. Proteins and proteomics.

Cofactors and metabolites as protein folding helpers in metabolic diseases.

João V. Rodrigues, C. Gomes, Bárbara J Henriques, 2013, Current topics in medicinal chemistry.

Riboflavin (vitamin B2) and mitochondrial energy

C. Gomes, Bárbara J Henriques, 2020 .

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

João V. Rodrigues, L. Vilarinho, C. Gomes, 2012, Biochimica et biophysica acta.

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

P. Bross, João V. Rodrigues, C. Gomes, 2009, Journal of Biological Chemistry.

Combined spectroscopic and calorimetric characterisation of rubredoxin reversible thermal transition

C. Gomes, L. M. Saraiva, Bárbara J Henriques, 2005, JBIC Journal of Biological Inorganic Chemistry.

Molecular and Clinical Investigations on Portuguese Patients with Multiple acyl-CoA Dehydrogenase Deficiency.

E. Martins, L. Vilarinho, C. Gomes, 2019, Current molecular medicine.

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

P. Bross, João V. Rodrigues, C. Gomes, 2011, Biochimica et biophysica acta.

Ethylmalonic Encephalopathy ETHE1 R163W/R163Q Mutations Alter Protein Stability and Redox Properties of the Iron Centre

V. Tiranti, P. Bross, João V. Rodrigues, 2014, PloS one.

Biophysical and Spectroscopic Methods for Monitoring Protein Misfolding and Amyloid Aggregation.

C. Gomes, Bárbara J Henriques, Joana S. Cristóvão, 2018, Methods in molecular biology.

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

O. Ilkayeva, C. Gomes, Michael B. Major, 2022, The Journal of biological chemistry.

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

P. Bross, C. Gomes, Bárbara J Henriques, 2011, FEBS letters.

Thermal Shift and Stability Assays of Disease-Related Misfolded Proteins Using Differential Scanning Fluorimetry.

C. Gomes, Bárbara J Henriques, Tânia G. Lucas, 2018, Methods in molecular biology.