Evidence map›Paper›PMID 41240228›Full record

ArticleMetabolic brain disease2025

Neuroprotective, antioxidant and anti-inflammatory effect of carnitine in patients with Maple syrup urine disease: branched-chain amino acids and branched-chain keto acids levels.

Júlia Girardi, Jéssica Lamberty Faverzani, Franciele Fátima Lopes, Angela Sitta, Daniella de Moura Coelho, Luísa Maria Bosquetti Tedesco, Natacha Dornelles, Moacir Wajner, Carmen Regla Vargas

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In one paragraph

Article in Metabolic brain disease, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Júlia GirardiPrograma de Pós-Graduação em Ciências Biológicas, Bioquímica Universidade Federal Do Rio Grande Do Sul, Rua Ramiro Barcelos, 2600, Porto Alegre, RS, CEP 90035-003, Brazil.ORCID 0000-0003-1214-2199
Jéssica Lamberty FaverzaniServiço de Genética Médica, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcellos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil. jelamberty@hotmail.com.ORCID 0000-0002-3695-6827
Franciele Fátima LopesServiço de Genética Médica, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcellos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil.ORCID 0000-0002-5022-3742
Angela SittaServiço de Genética Médica, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcellos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil.ORCID 0000-0002-4801-7202
Daniella de Moura CoelhoServiço de Genética Médica, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcellos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil.ORCID 0000-0002-3028-1849
Luísa Maria Bosquetti TedescoFaculdade de Farmácia, Universidade Federal do Rio Grande do Sul, UFRGS, Avenida Ipiranga, 2752, Porto Alegre, RS, CEP 90610-000, Brazil.ORCID 0000-0002-5223-1041
Natacha DornellesFaculdade de Farmácia, Universidade Federal do Rio Grande do Sul, UFRGS, Avenida Ipiranga, 2752, Porto Alegre, RS, CEP 90610-000, Brazil.ORCID 0009-0009-4799-7569
Moacir WajnerServiço de Genética Médica, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcellos, 2350, Porto Alegre, RS, CEP 90035-003, Brazil.
Carmen Regla VargasPrograma de Pós-Graduação em Ciências Biológicas, Bioquímica Universidade Federal Do Rio Grande Do Sul, Rua Ramiro Barcelos, 2600, Porto Alegre, RS, CEP 90035-003, Brazil. crvargas@hcpa.edu.br.ORCID 0000-0002-2135-0654

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Maple syrup urine disease (MSUD) is a genetic disorder of the metabolism of branched-chain amino acids (BCAAs). We investigate the effects of treatment with dietary restriction and carnitine supplementation in MSUD patients. During treatment, patients were placed on a restricted diet and received a semi-synthetic formula enriched with carnitine. Our results revealed that treated patients showed elevated levels of neurodegeneration biomarkers (BDNF and PDGF-BB), while levels of TBARS and isoprostanes, indicators of lipid oxidative damage, were significantly decreased compared to diagnostic group. We observed an increase in L-carnitine levels after treatment, which suggests a positive response to supplementation. BCAAs and branched-chain α-keto acid dehydrogenase levels were elevated at diagnosis but decreased after treatment, indicating therapeutic efficacy. Our results highlight the importance of carnitine supplementation in the treatment of MSUD patients, possibly mitigating the neurological and metabolic involvement of the disease, providing valuable insights to optimize therapy and improve clinical outcomes.

Indexed as

Amino Acids, Branched-ChainAnti-Inflammatory AgentsAntioxidantsCarnitineKeto AcidsMaple Syrup Urine DiseaseNeuroprotective AgentsAdolescentAdultChildChild, PreschoolDietary SupplementsFemaleHumansMaleOxidative StressAmino Acids, Branched-ChainAnti-Inflammatory AgentsAntioxidantsCarnitineKeto AcidsNeuroprotective AgentsBrain-derived neurotrophic factor (BDNF)CarnitineMaple syrup urine diseaseOxidative damagePlatelet-derived growth factor (PDGF-BB)

Identifiers

PMID41240228

What Socratic holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.