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Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation

Overview of attention for article published in Metabolic Brain Disease, May 2015
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Title
Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation
Published in
Metabolic Brain Disease, May 2015
DOI 10.1007/s11011-015-9686-9
Pubmed ID
Authors

Caroline Paula Mescka, Gilian Guerreiro, Bruna Donida, Desirèe Marchetti, Carlos Alberto Yasin Wayhs, Graziela Schimitt Ribas, Adriana Simon Coitinho, Moacir Wajner, Carlos Severo Dutra-Filho, Carmen Regla Vargas

Abstract

Maple Syrup Urine Disease (MSUD) is a metabolic disorder caused by a severe deficiency of the branched-chain α-keto acid dehydrogenase complex activity which leads to the accumulation of branched-chain amino acids (BCAA) leucine (Leu), isoleucine and valine and their respective α-keto-acids in body fluids. The main symptomatology presented by MSUD patients includes ketoacidosis, failure to thrive, poor feeding, apnea, ataxia, seizures, coma, psychomotor delay and mental retardation, but, the neurological pathophysiologic mechanisms are poorly understood. The treatment consists of a low protein diet and a semi-synthetic formula restricted in BCAA and supplemented with essential amino acids. It was verified that MSUD patients present L-carnitine (L-car) deficiency and this compound has demonstrated an antioxidant and anti-inflammatory role in metabolic diseases. Since there are no studies in the literature reporting the inflammatory profile of MSUD patients and the L-car role on the inflammatory response in this disorder, the present study evaluates the effect of L-car supplementation on plasma inflammatory cytokines interleukin-1β (IL-1β), interleukin-6 (IL-6), interferon-gamma (INF-ɣ), and a correlation with malondialdehyde (MDA), as a marker of oxidative damage, and with free L-car plasma levels in treated MSUD patients. Significant increases of IL-1β, IL-6, and INF-ɣ were observed before the treatment with L-car. Moreover, there is a negative correlation between all cytokines tested and L-car concentrations and a positive correlation among the MDA content and IL-1β and IL-6 values. Our data show that L-car supplementation can improve cellular defense against inflammation and oxidative stress in MSUD patients and may represent an additional therapeutic approach to the patients affected by this disease.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 45 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 45 100%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 9 20%
Student > Ph. D. Student 7 16%
Student > Postgraduate 6 13%
Student > Doctoral Student 4 9%
Other 3 7%
Other 6 13%
Unknown 10 22%
Readers by discipline Count As %
Medicine and Dentistry 15 33%
Biochemistry, Genetics and Molecular Biology 4 9%
Neuroscience 3 7%
Psychology 3 7%
Nursing and Health Professions 2 4%
Other 5 11%
Unknown 13 29%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 25 June 2016.
All research outputs
#17,758,791
of 22,807,037 outputs
Outputs from Metabolic Brain Disease
#664
of 1,053 outputs
Outputs of similar age
#180,519
of 267,399 outputs
Outputs of similar age from Metabolic Brain Disease
#13
of 24 outputs
Altmetric has tracked 22,807,037 research outputs across all sources so far. This one is in the 19th percentile – i.e., 19% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,053 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 5.3. This one is in the 32nd percentile – i.e., 32% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 267,399 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 27th percentile – i.e., 27% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 24 others from the same source and published within six weeks on either side of this one. This one is in the 37th percentile – i.e., 37% of its contemporaries scored the same or lower than it.