Increased levels of 4HNE-protein plasma adducts in Rett syndrome

Alessandra Pecorelli, Lucia Ciccoli, Cinzia Signorini, Silvia Leoncini, Anna Giardini, Maurizio D'Esposito, Stefania Filosa, Joussef Hayek, Claudio De Felice, Giuseppe Valacchi

Research output: Contribution to journalArticle

Abstract

Objective: Rett syndrome (RTT) is a neurological disorder and a leading cause of mental retardation in females. It is caused by mutations in methyl-CpG-binding protein 2 (MeCP2) gene and more rarely in cyclin-dependent kinase-like 5 (CDKL5) and forkhead box protein G1 (FOXG1) genes. Increased oxidative stress (OS) has been documented in MeCP2-RTT patients. Here, we evaluated the levels of 4-hydroxynonenal plasma protein adducts (4HNE-PAs) in MeCP2-, CDKL5-, and FOXG1-RTT and in their clinical variants. Design and methods: 4HNE-PAs were determined by Western blot in plasma from healthy subjects and RTT patients. Results: 4HNE-PAs levels were increased in MeCP2- and CDKL5-related RTT but not in FOXG1-related RTT. Conclusion: These results showed that OS is present in RTT clinical variants and could play a key role in RTT pathogenesis. Under the OS point of view FOXG1-related RTT appears to be distinct from the MeCP2/CDKL5, suggesting a distinct mechanism involved in its pathogenesis.

Original languageEnglish
Pages (from-to)368-371
Number of pages4
JournalClinical Biochemistry
Volume44
Issue number5-6
DOIs
Publication statusPublished - Apr 2011

Fingerprint

Methyl-CpG-Binding Protein 2
Rett Syndrome
Cyclin-Dependent Kinases
Blood Proteins
Oxidative stress
Cyclin-Dependent Kinase 5
Genes
Forkhead Transcription Factors
Oxidative Stress
Plasmas
Nervous System Diseases
Intellectual Disability
Healthy Volunteers
4-hydroxy-2-nonenal
Western Blotting

Keywords

  • 4-Hydroxynonenal
  • CDKL5
  • FOXG1
  • MeCP2
  • Oxidative stress
  • Rett syndrome

ASJC Scopus subject areas

  • Clinical Biochemistry

Cite this

Pecorelli, A., Ciccoli, L., Signorini, C., Leoncini, S., Giardini, A., D'Esposito, M., ... Valacchi, G. (2011). Increased levels of 4HNE-protein plasma adducts in Rett syndrome. Clinical Biochemistry, 44(5-6), 368-371. https://doi.org/10.1016/j.clinbiochem.2011.01.007

Increased levels of 4HNE-protein plasma adducts in Rett syndrome. / Pecorelli, Alessandra; Ciccoli, Lucia; Signorini, Cinzia; Leoncini, Silvia; Giardini, Anna; D'Esposito, Maurizio; Filosa, Stefania; Hayek, Joussef; De Felice, Claudio; Valacchi, Giuseppe.

In: Clinical Biochemistry, Vol. 44, No. 5-6, 04.2011, p. 368-371.

Research output: Contribution to journalArticle

Pecorelli, A, Ciccoli, L, Signorini, C, Leoncini, S, Giardini, A, D'Esposito, M, Filosa, S, Hayek, J, De Felice, C & Valacchi, G 2011, 'Increased levels of 4HNE-protein plasma adducts in Rett syndrome', Clinical Biochemistry, vol. 44, no. 5-6, pp. 368-371. https://doi.org/10.1016/j.clinbiochem.2011.01.007
Pecorelli, Alessandra ; Ciccoli, Lucia ; Signorini, Cinzia ; Leoncini, Silvia ; Giardini, Anna ; D'Esposito, Maurizio ; Filosa, Stefania ; Hayek, Joussef ; De Felice, Claudio ; Valacchi, Giuseppe. / Increased levels of 4HNE-protein plasma adducts in Rett syndrome. In: Clinical Biochemistry. 2011 ; Vol. 44, No. 5-6. pp. 368-371.
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