Literature DB >> 18088602

Maple syrup urine disease in treated patients: biochemical and oxidative stress profiles.

Alethéa G Barschak1, Christiane Marchesan, Angela Sitta, Marion Deon, Roberto Giugliani, Moacir Wajner, Carmen Regla Vargas.   

Abstract

OBJECTIVE: The objective of this study was to evaluate and correlate the biochemical and oxidative stress profiles in MSUD patients during the dietary treatment. DESIGN AND METHODS: Plasma samples from treated MSUD patients were used to evaluate the biochemical profile and oxidative stress parameters.
RESULTS: It was observed that glucose, total cholesterol, albumin and creatinine are reduced and that aspartate aminotransferase and lactate dehydrogenase activities are increased in plasma from MSUD patients under treatment. Besides, it was verified an increase of thiobarbituric acid-reactive species (TBARS) and a decrease of total antioxidant reactivity (TAR).
CONCLUSIONS: Our results suggest that oxidative stress occurs in treated MSUD patients and that dietary treatment and clinical conditions associated to the disease can lead to biochemical alterations in these patients.

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Year:  2007        PMID: 18088602     DOI: 10.1016/j.clinbiochem.2007.11.015

Source DB:  PubMed          Journal:  Clin Biochem        ISSN: 0009-9120            Impact factor:   3.281


  11 in total

1.  Neuroprotective Effect of Creatine and Pyruvate on Enzyme Activities of Phosphoryl Transfer Network and Oxidative Stress Alterations Caused by Leucine Administration in Wistar Rats.

Authors:  Elenara Rieger; Itiane Diehl de Franceschi; Thales Preissler; Clovis Milton Duval Wannmacher
Journal:  Neurotox Res       Date:  2017-06-13       Impact factor: 3.911

2.  Catabolic Defect of Branched-Chain Amino Acids Promotes Heart Failure.

Authors:  Haipeng Sun; Kristine C Olson; Chen Gao; Domenick A Prosdocimo; Meiyi Zhou; Zhihua Wang; Darwin Jeyaraj; Ji-Youn Youn; Shuxun Ren; Yunxia Liu; Christoph D Rau; Svati Shah; Olga Ilkayeva; Wen-Jun Gui; Noelle S William; R Max Wynn; Christopher B Newgard; Hua Cai; Xinshu Xiao; David T Chuang; Paul Christian Schulze; Christopher Lynch; Mukesh K Jain; Yibin Wang
Journal:  Circulation       Date:  2016-04-08       Impact factor: 29.690

3.  Investigation of L - Carnitine Concentrations in Treated Patients with Maple Syrup Urine Disease.

Authors:  Burcu Kumru; Burcu Oztürk Hismi
Journal:  J Pediatr Genet       Date:  2019-05-28

4.  Acute and long-term effects of intracerebroventricular administration of α-ketoisocaproic acid on oxidative stress parameters and cognitive and noncognitive behaviors.

Authors:  Luciane Taschetto; Giselli Scaini; Hugo G Zapelini; Ândrea C Ramos; Giulia Strapazzon; Vanessa M Andrade; Gislaine Z Réus; Monique Michels; Felipe Dal-Pizzol; João Quevedo; Patrícia F Schuck; Gustavo C Ferreira; Emilio L Streck
Journal:  Metab Brain Dis       Date:  2017-05-27       Impact factor: 3.584

5.  Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD).

Authors:  Kristen J Skvorak; Elizabeth J Hager; Erland Arning; Teodoro Bottiglieri; Harbhajan S Paul; Stephen C Strom; Gregg E Homanics; Qin Sun; Erwin E W Jansen; Cornelis Jakobs; William J Zinnanti; K Michael Gibson
Journal:  Biochim Biophys Acta       Date:  2009-08-19

6.  Protein phosphatase 2Cm is a critical regulator of branched-chain amino acid catabolism in mice and cultured cells.

Authors:  Gang Lu; Haipeng Sun; Pengxiang She; Ji-Youn Youn; Sarah Warburton; Peipei Ping; Thomas M Vondriska; Hua Cai; Christopher J Lynch; Yibin Wang
Journal:  J Clin Invest       Date:  2009-06       Impact factor: 14.808

7.  Apoptotic signaling pathways induced by acute administration of branched-chain amino acids in an animal model of maple syrup urine disease.

Authors:  Thais C Vilela; Giselli Scaini; Camila B Furlanetto; Matheus A B Pasquali; João Paulo A Santos; Daniel P Gelain; José Cláudio F Moreira; Patrícia F Schuck; Gustavo C Ferreira; Emilio L Streck
Journal:  Metab Brain Dis       Date:  2016-08-11       Impact factor: 3.584

Review 8.  Neurological damage in MSUD: the role of oxidative stress.

Authors:  Angela Sitta; Graziela S Ribas; Caroline P Mescka; Alethéa G Barschak; Moacir Wajner; Carmen R Vargas
Journal:  Cell Mol Neurobiol       Date:  2013-11-13       Impact factor: 5.046

9.  Maple syrup urine disease in Brazilian patients: variants and clinical phenotype heterogeneity.

Authors:  Ana Vitoria Barban Margutti; Wilson Araújo Silva; Daniel Fantozzi Garcia; Greice Andreotti de Molfetta; Adriana Aparecida Marques; Tatiana Amorim; Vânia Mesquita Gadelha Prazeres; Raquel Tavares Boy da Silva; Irene Kazue Miura; João Seda Neto; Emerson de Santana Santos; Mara Lúcia Schmitz Ferreira Santos; Charles Marques Lourenço; Tássia Tonon; Fernanda Sperb-Ludwig; Carolina Fischinger Moura de Souza; Ida Vanessa Döederlein Schwartz; José Simon Camelo
Journal:  Orphanet J Rare Dis       Date:  2020-11-01       Impact factor: 4.123

10.  Brain-blood amino acid correlates following protein restriction in murine maple syrup urine disease.

Authors:  Kara R Vogel; Erland Arning; Brandi L Wasek; Sterling McPherson; Teodoro Bottiglieri; K Michael Gibson
Journal:  Orphanet J Rare Dis       Date:  2014-05-08       Impact factor: 4.123

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