Universitat de Lleida
    • English
    • català
    • español
  • English 
    • English
    • català
    • español
  • Login
Repositori Obert UdL
View Item 
  •   Home
  • Recerca
  • Ciències Mèdiques Bàsiques
  • Articles publicats (Ciències Mèdiques Bàsiques)
  • View Item
  •   Home
  • Recerca
  • Ciències Mèdiques Bàsiques
  • Articles publicats (Ciències Mèdiques Bàsiques)
  • View Item
JavaScript is disabled for your browser. Some features of this site may not work without it.

Protein oxidation in Huntington disease affects energy production and vitamin B6 metabolism

Thumbnail
View/Open
015962.pdf (1.218Mb)
Sol·licita una còpia
Issue date
2010
Author
Sorolla Bardají, Maria Alba
Rodríguez Colman, Maria José
Tamarit Sumalla, Jordi
Ortega, Zaira
Lucas, José J.
Ferrer, Isidre
Ros Salvador, Joaquim
Cabiscol Català, Elisa
Suggested citation
Sorolla Bardají, Maria Alba; Rodríguez Colman, Maria José; Tamarit Sumalla, Jordi; Ortega, Zaira; Lucas, José J.; Ferrer, Isidre; ... Cabiscol Català, Elisa. (2010) . Protein oxidation in Huntington disease affects energy production and vitamin B6 metabolism. Free Radical Biology and Medicine, 2010, vol. 49, núm. 4, p. 612-621. https://doi.org/10.1016/j.freeradbiomed.2010.05.016.
Impact


Web of Science logo    citations in Web of Science

Scopus logo    citations in Scopus

Google Scholar logo  Google Scholar
Share
Export to Mendeley
Metadata
Show full item record
Abstract
Huntington disease (HD) is an inherited neurodegenerative disorder that initially affects the striatum and progressively the cortex. Oxidative stress in HD has been described as important to disease progression. In this study, protein carbonylation, used as a marker of protein oxidation, was analyzed in human brain striatum. A comparison of HD samples tomatched controls identified 13 carbonylated proteins, including enzymes involved in the glycolytic pathway and mitochondrial proteins related to ATP production. Oxidation of the mitochondrial enzymes resulted in decreased catalytic activity, in good agreement with the energy deficiency observed in HD. Wealso found carbonylation of pyridoxal kinase and antiquitin 1, both involved in themetabolismof pyridoxal 5- phosphate, the active formof vitamin B6. The Tet/HD94 conditionalmousemodel allowed us to demonstrate that increased carbonylation in striatum is dependent on mutant huntingtin expression. As in humans, pyridoxal kinase showed decreased levels and was highly carbonylated in the gene-on mice; these modifications were reverted in the gene-offmice. Wehypothesize that both pyridoxal kinase and antiquitin 1 oxidation could result in decreased pyridoxal 5-phosphate availability necessary as a cofactor in transaminations, synthesis of glutathione, and synthesis of GABA and dopamine, two neurotransmitters that play a key role in HD pathology.
URI
http://hdl.handle.net/10459.1/57169
DOI
https://doi.org/10.1016/j.freeradbiomed.2010.05.016
Is part of
Free Radical Biology and Medicine, 2010, vol. 49, núm. 4, p. 612-621
European research projects
Collections
  • Articles publicats (IRBLleida) [755]
  • Publicacions de projectes de recerca del Plan Nacional [2172]
  • Articles publicats (Ciències Mèdiques Bàsiques) [474]
  • Articles publicats (Grup de Recerca en Estrès Cel·lular i Supervivència en Models Eucariotes) [51]

Related items

Showing items related by title, author, creator and subject.

  • Sir2 is induced by oxidative stress in a yeast model of Huntington disease and its activation reduces protein aggregation 

    Sorolla Bardají, Maria Alba; Nierga, Clara; Rodríguez Colman, Maria José; Reverter Branchat, Gemma; Arenas, Alicia; Tamarit Sumalla, Jordi; Ros Salvador, Joaquim; Cabiscol Català, Elisa (Elsevier, 2011)
    Huntington disease (HD) is a neurodegenerative disorder caused by expansion of CAG trinucleotide repeats, leading to an elongated polyglutamine sequence (polyQ) in the huntingtin protein. Misfolding of mutant polyQ ...
  • Protein oxidation in Huntington disease 

    Sorolla Bardají, Maria Alba; Rodríguez Colman, Maria José; Vall-llaura Espinosa, Núria; Tamarit Sumalla, Jordi; Ros Salvador, Joaquim; Cabiscol Català, Elisa (Wiley, 2012-05)
    Huntington disease (HD) is an inherited neurodegenerative disorder caused by expansion of CAG repeats in the huntingtin gene, affecting initially the striatum and progressively the cortex. Oxidative stress, and consequent ...
  • Proteomic and oxidative stress analysis in human brain samples of Huntington disease 

    Sorolla Bardají, Maria Alba; Reverter Branchat, Gemma; Tamarit Sumalla, Jordi; Ferrer, Isidre; Ros Salvador, Joaquim; Cabiscol Català, Elisa (Elsevier, 2008)
    Huntington disease (HD) is a neurodegenerative disorder caused by expansion of CAG repeats in exon 1 of the huntingtin gene, affecting initially the striatum and progressively the cortex. This work reports a proteomic analysis ...

Contact Us | Send Feedback | Legal Notice
© 2021 BiD. Universitat de Lleida
Metadata subjected to 
 

 

Browse

All of the repositoryCommunities & CollectionsBy Issue DateAuthorsTitlesSubjectsThis CollectionBy Issue DateAuthorsTitlesSubjects

Statistics

View Usage Statistics

D'interès

Política institucional d'accés obertDiposita les teves publicacionsDiposita dades de recercaSuport a la recerca

Contact Us | Send Feedback | Legal Notice
© 2021 BiD. Universitat de Lleida
Metadata subjected to