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Cited by 15Genetic Neurodegenerative DiseasesMetabolomics and Mass Spectrometry StudiesMetabolism and Genetic Disorders

Altered Plasma Acylcarnitines and Amino Acids Profile in Spinocerebellar Ataxia Type 7

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Nambo-Venegas, Rafael, Valdez-Vargas, Claudia, Cisneros, Bulmaro, Palacios-González, Berenice, Vela-Amieva, Marcela, Ibarra-González, Isabel, Cerecedo-Zapata, César M., Martínez-Cruz, Emilio +3 more·2020·Biomolecules
DOI 10.3390/biom10030390PMC PMC7175318
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Abstract

Spinocerebellar ataxia type 7 (SCA7), a neurodegenerative disease characterized by cerebellar ataxia and retinal degeneration, is caused by an abnormal CAG repeat expansion in the ATXN7 gene coding region. The onset and severity of SCA7 are highly variable between patients, thus identification of sensitive biomarkers that accurately diagnose the disease and monitoring its progression are needed. With the aim of identified SCA7-specific metabolites with clinical relevance, we report for the first time, to the best of our knowledge, a metabolomics profiling of circulating acylcarnitines and amino acids in SCA7 patients. We identified 21 metabolites with altered levels in SCA7 patients and determined two different sets of metabolites with diagnostic power. The first signature of metabolites (Valine, Leucine, and Tyrosine) has the ability to discriminate between SCA7 patients and healthy controls, while the second one (Methionine, 3-hydroxytetradecanoyl-carnitine, and 3-hydroxyoctadecanoyl-carnitine) possess the capability to differentiate between early-onset and adult-onset patients, as shown by the multivariate model and ROC analyses. Furthermore, enrichment analyses of metabolic pathways suggest alterations in mitochondrial function, energy metabolism, and fatty acid beta-oxidation in SCA7 patients. In summary, circulating SCA7-specific metabolites identified in this study could serve as effective predictors of SCA7 progression in the clinics, as they are sampled in accessible biofluid and assessed by a relatively simple biochemical assay.

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  • By analyzing hydrolysates of native and modified protein samples, amino acid composition can provide information about the loss of parent proteins and the formation of products.

    “By analyzing hydrolysates of native and modified protein samples, the amino acid composition can provide information about the loss of parent proteins, but also the formation of products [101].”

    From Protein oxidation - Formation mechanisms, detection and relevance as biomarkers in human diseases (2021) · cites this paper as [101]

    The Metabolomics Analysis section describes measuring amino acids in blood plasma, and Results §3.2 reports differences between SCA7 patients and controls. Neither addresses hydrolysates of native and modified proteins or whether amino acid composition reveals loss of parent proteins and formation of products. Searches for those terms and for protein hydrolysis found no supporting passage. (LLM, conf=0.76)

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References 55 · Cited by 15

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Authors
Nambo-Venegas, R., Valdez-Vargas, C., Cisneros, B., Palacios-González, B., Vela-Amieva, M., Ibarra-González, I., Cerecedo-Zapata, C. M., Martínez-Cruz, E., Cortés, H., Reyes-Grajeda, J. P., & Magaña, J. J.
Affiliations
  • Laboratory of Chronic Diseases Biochemistry, National Genomics Medicine Institute (INMEGEN), Mexico City 14610, Mexico
  • Instituto Nacional de Rehabilitación (MX)
  • Centro de Investigación y de Estudios Avanzados del Instituto Politécnico Nacional (MX)
  • Scientific Bonding Unit, Medicine Faculty UNAM-INMEGEN, Mexico City 14610, Mexico
  • Instituto Nacional de Pediatria (MX)
  • Institute of Biomedical Research, IIB-UNAM, Mexico City 04510, Mexico
  • Rehabilitation and Special Education Center of Veracruz (CRISVER-DIF), Xalapa 91097, Veracruz, Mexico
Corresponding author
Magaña, Jonathan J.
ORCID
Venue
Biomolecules
Publisher
MDPI AG
Published
2020-03-03
Volume
10
Issue
3
Pages
390
ISSN
2218-273X (ISSN-L)
DOI
10.3390/biom10030390
PMC
PMC7175318
OpenAlex
W3009588034
Cited by
15
References
55
Citations by year
2026: 3 · 2025: 1 · 2024: 2 · 2023: 3 · 2022: 2 · 2021: 3 · 2020: 1
FWCI
1.03
Type
journal article
Language
en
Open access
Licence
Locations
  • Biomolecules (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
CONACyT (258043)
Topics
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Metabolomics and Mass Spectrometry Studies
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Keywords
spinocerebellar ataxia type 7, acylcarnitines, amino acids, plasma metabolomics, biomarkers, valine, leucine, tyrosine, methionine, disease progression, mitochondrial function, energy metabolism, fatty acid beta-oxidation
MeSH terms
Adult, Amino Acids, Carnitine, Female, Humans, Male, Middle Aged, Biomarkers, Spinocerebellar Ataxias

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