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Wnt signaling alteration in the spinal cord of Amyotrophic Lateral Sclerosis transgenic mice : special focus on Frizzled-5 cellular expression pattern
González-Fernández, Carlos (Hospital Nacional de Parapléjicos (Toledo))
Mancuso, Renzo (Universitat Autònoma de Barcelona. Institut de Neurociències)
Del Valle i Macià, Jaume (Universitat Autònoma de Barcelona. Institut de Neurociències)
Navarro, X. (Xavier) (Universitat Autònoma de Barcelona. Departament de Biologia Cel·lular, de Fisiologia i d'Immunologia)
Rodríguez, Francisco Javier (Hospital Nacional de Parapléjicos (Toledo))

Date: 2016
Abstract: Background: Amyotrophic lateral sclerosis is a chronic neurodegenerative disease characterized by progressive paralysis due to degeneration of motor neurons by unknown causes. Recent evidence shows that Wnt signaling is involved in neurodegenerative processes, including Amyotrophic Lateral Sclerosis. However, to date, little is known regarding the expression of Wnt signaling components in this fatal condition. In the present study we used transgenic SOD1G93A mice to evaluate the expression of several Wnt signaling components, with special focus on Frizzled-5 cellular expression alteration along disease progression. Findings: Based on previous studies demonstrating the expression of Wnts and their transcriptional regulation during Amyotrophic lateral sclerosis development, we have analyzed the mRNA expression of several Wnt signaling components in the spinal cord of SOD1G93A transgenic mice at different stages of the disease by using real time quantitative PCR analysis. Strikingly, one of the molecules that seemed not to be altered at mRNA level, Frizzled-5, showed a clear up-regulation at late stages in neurons, as evidenced by immunofluorescence assays. Moreover, increased Frizzled-5 appears to correlate with a decrease in NeuN signal in these cells, suggesting a correlation between neuronal affectation and the increased expression of this receptor. Conclusions: Our data suggest the involvement of Wnt signaling pathways in the pathophysiology of Amyotrophic Lateral Sclerosis and, more specifically, the implication of Frizzled-5 receptor in the response of neuronal cells against neurodegeneration. Nevertheless, further experimental studies are needed to shed light on the specific role of Frizzled-5 and the emerging but increasing Wnt family of proteins research field as a potential target for this neuropathology.
Note: Altres ajuts: Fundació La Marato-TV3 (TV3201428-10)
Note: Número d'acord de subvenció ISCIII/FIS/PI12/2895
Note: Número d'acord de subvenció AGAUR/2014/SGR-201
Rights: Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original. Creative Commons
Language: Anglès.
Document: article ; recerca ; publishedVersion
Subject: Spinal cord ; Mouse models ; Neurons ; Wnt signaling cascade ; Amyotrophic lateral sclerosis ; Astrocytes ; Gene expression ; Protein expression
Published in: PLoS One, Vol. 11, No 5 (May 2016) , p. e0155867, ISSN 1932-6203

DOI: 10.1371/journal.pone.0155867
PMID: 27192435

17 p, 38.6 MB

The record appears in these collections:
Research literature > UAB research groups literature > Research Centres and Groups (scientific output) > Health sciences and biosciences > Institut de Neurociències (INc)
Articles > Research articles
Articles > Published articles

 Record created 2016-06-16, last modified 2018-10-28

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