Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of L-carnitine supplementation

International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience
Graziela S RibasCarmen R Vargas

Abstract

Disorders of propionate metabolism are autosomal recessive diseases clinically characterized by acute metabolic crises in the neonatal period and long-term neurological deficits whose pathophysiology is not completely established. There are increasing evidences demonstrating antioxidant properties for L-carnitine, which is used in the treatment of propionic and methylmalonic acidemias to increase the excretion of organic acids accumulated in tissues and biological fluids of the affected patients. In this work we aimed to evaluate lipid (malondialdehyde content) and protein (carbonyl formation and sulfhydryl oxidation) oxidative damage in plasma from patients with propionic and methylmalonic acidemias at the moment of diagnosis and during treatment with L-carnitine. We also correlated the parameters of oxidative damage with plasma total, free and esterified L-carnitine levels. We found a significant increase of malondialdehyde and carbonyl groups, as well as a reduction of sulfhydryl groups in plasma of these patients at diagnosis compared to controls. Furthermore, patients under treatment presented a marked reduction of the content of protein carbonyl groups, similar to controls, and malondialdehyde content in relation to patie...Continue Reading

References

Jan 1, 1991·Journal of Inherited Metabolic Disease·G F HoffmannH J Bremer
Jan 1, 1990·Methods in Enzymology·R L LevineE R Stadtman
Jan 1, 1990·Methods in Enzymology·H Esterbauer, K H Cheeseman
Mar 15, 1990·Clinica Chimica Acta; International Journal of Clinical Chemistry·C de SousaR A Chalmers
Jul 25, 1997·Clinica Chimica Acta; International Journal of Clinical Chemistry·D MoyanoC Sierra
Aug 5, 2000·Cell Biology and Toxicology·A VanellaM L Barcellona
Mar 23, 2002·The Journals of Gerontology. Series A, Biological Sciences and Medical Sciences·P Juliet Arockia Rani, C Panneerselvam
Jun 19, 2002·Seminars in Neonatology : SN·H Ogier de Baulny, J M Saudubray
Feb 5, 2003·The International Journal of Biochemistry & Cell Biology·Michele Rechia FigheraCarlos Fernando Mello
May 17, 2003·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Charles Hoppel
Jul 11, 2003·Annals of the New York Academy of Sciences·Ashraf VirmaniSyed Ali
Sep 16, 2005·Epilepsy Research·Marinei Cristina Pereira RibeiroCarlos Fernando Mello
Oct 29, 2005·Life Sciences·Ilhami Gülçin
Dec 6, 2005·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Leticia F PettenuzzoMoacir Wajner
Feb 14, 2006·Pharmacology, Biochemistry, and Behavior·Luiz Fernando Freire RoyesCarlos Fernando Mello
Feb 17, 2006·Clinica Chimica Acta; International Journal of Clinical Chemistry·Anusuya Devi MuthuswamyPanneerselvam Chinnakannu
Apr 8, 2006·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Federica DeodatoCarlo Dionisi-Vici
Jun 8, 2006·Neurochemical Research·Laura O SaadRoger F Castilho
Sep 12, 2006·Neurochemistry International·Ana Flávia FurianLuiz Fernando Freire Royes
Sep 26, 2006·Neuroscience Letters·Flávia Karine RigoCarlos Fernando Mello
Dec 5, 2006·The Journal of Nutritional Biochemistry·Lucía Gómez-AmoresCarmen M Vázquez
Sep 11, 2007·Pharmacological Research : the Official Journal of the Italian Pharmacological Society·Nurettin TastekinMevlut Ture
Nov 27, 2007·Free Radical Biology & Medicine·Chutima MatayatsukBarry Halliwell
Jan 24, 2008·Journal of Inherited Metabolic Disease·S R MirandolaR F Castilho
May 22, 2008·Journal of Pediatric Nursing·Lori Van Gosen
Sep 25, 2008·Cellular and Molecular Neurobiology·Angela SittaCarmen R Vargas
Nov 11, 2008·Clinica Chimica Acta; International Journal of Clinical Chemistry·Moacir WajnerCarmen Regla Vargas
Mar 3, 2009·Alcohol·Agnieszka Augustyniak, Elzbieta Skrzydlewska

❮ Previous
Next ❯

Citations

Feb 4, 2011·Journal of Inherited Metabolic Disease·Ina KnerrK Michael Gibson
Apr 26, 2011·Cellular and Molecular Neurobiology·Graziela S RibasCarmen R Vargas
Nov 14, 2013·Cellular and Molecular Neurobiology·Angela SittaCarmen R Vargas
Feb 19, 2014·Gene·Camila Simioni VanzinCarmen Regla Vargas
Nov 10, 2012·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Caroline Paula MesckaCarmen Regla Vargas
Feb 15, 2015·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Gilian GuerreiroCarmen Regla Vargas
Jul 15, 2015·Cellular and Molecular Neurobiology·Marion DeonCarmen Vargas
Apr 14, 2015·Mutation Research·Caroline Paula MesckaCarmen Regla Vargas
Jan 6, 2015·Molecular and Cellular Biochemistry·Mariana Dos Santos MelloCarmen Regla Vargas
Jan 24, 2016·Journal of Neurochemistry·Alexandre Umpierrez AmaralMoacir Wajner
May 17, 2017·Neurochemical Research·Gustavo C Ferreira, Mary C McKenna
May 11, 2018·Oxidative Medicine and Cellular Longevity·Eva RichardLourdes R Desviat
Mar 28, 2019·Nature Reviews. Neurology·Moacir Wajner
Mar 8, 2011·Metabolic Brain Disease·Caroline MesckaCarlos S Dutra-Filho
Dec 1, 2018·Metabolic Brain Disease·Mehmet Serif CanseverCigdem Aktuglu-Zeybek
Jan 29, 2021·Frontiers in Aging Neuroscience·Jessica KillingsworthR Douglas Shytle
Feb 9, 2021·Journal of Diabetes and Metabolic Disorders·Morteza AliashrafiSeyed Davar Siadat
Apr 4, 2021·Journal of Clinical Medicine·Alina KępkaNapoleon Waszkiewicz
Oct 26, 2020·Archives of Biochemistry and Biophysics·Moacir WajnerAlexandre Umpierrez Amaral

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

© 2021 Meta ULC. All rights reserved