Phosphorylated Smad2/3 immunoreactivity in sporadic and familial amyotrophic lateral sclerosis and its mouse model

Acta Neuropathologica
Masataka NakamuraHirofumi Kusaka

Abstract

Phosphorylated Smad2/3 (pSmad2/3), the central mediators of transforming growth factor (TGF)-beta signaling, were recently identified in tau-positive inclusions in certain neurodegenerative disorders. To clarify whether the localization of pSmad2/3 is altered in amyotrophic lateral sclerosis (ALS), we immunohistochemically examined spinal cords from sporadic ALS (SALS), from familial ALS (FALS) patients with the A4V mutation in their Cu/Zn superoxide dismutase (SOD1) gene, and from G93A mutant SOD1 transgenic (mSOD1 Tg) mice. In control spinal cords, pSmad2/3 immunoreactivity was observed exclusively in neuronal and glial nuclei. In SALS and FALS patients the nuclei showed increased immunoreactivity for pSmad2/3. Noticeably, round hyaline inclusions (RHIs) and skein-like inclusions of SALS patients were immunoreactive for pSmad2/3. Double immunofluorescence staining for pSmad2/3 and transactive response-DNA-binding protein (TDP)-43 revealed co-localization of these proteins within RHIs. In contrast, Bunina bodies in SALS and Lewy body-like hyaline inclusions (LBHIs) in FALS were devoid of labeling for pSmad2/3. Similarly, in the mSOD1 Tg mice pSmad2/3 immunoreactivity was increased in the nuclei, while LBHIs were not labeled. T...Continue Reading

References

Feb 14, 1998·Progress in Neurobiology·K C FlandersC F Lippa
Oct 10, 2002·Acta Neurologica Scandinavica·K HouiK Inoue
May 8, 2004·Trends in Biochemical Sciences·Peter ten Dijke, Caroline S Hill
Jan 18, 2006·Journal of Neuropathology and Experimental Neurology·Lynette G SheffieldSuzanne S Mirra
Nov 1, 2006·The European Journal of Neuroscience·Uwe UeberhamThomas Arendt
Nov 3, 2006·The Journal of Clinical Investigation·Ina TesseurTony Wyss-Coray
Dec 22, 2006·Biochimica Et Biophysica Acta·Lan Xu
Feb 7, 2007·Journal of Neuropathology and Experimental Neurology·Katy A Chalmers, Seth Love
Jun 15, 2007·Acta Neuropathologica·Dennis W DicksonCatalina Amador-Ortiz
Nov 15, 2007·Nature Reviews. Molecular Cell Biology·Bernhard Schmierer, Caroline S Hill

❮ Previous
Next ❯

Citations

Mar 10, 2009·Journal of Neurology·Felix GeserJohn Q Trojanowski
Jan 28, 2010·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Felix GeserJohn Q Trojanowski
Jan 27, 2012·Rinshō shinkeigaku = Clinical neurology·Masahisa KatsunoGen Sobue
Jun 14, 2008·Expert Opinion on Biological Therapy·Casey CookLeonard Petrucelli
Mar 23, 2012·Neuropathology and Applied Neurobiology·Masataka NakamuraHirofumi Kusaka
Sep 8, 2010·Neuropathology and Applied Neurobiology·M NakamuraH Kusaka
Jul 19, 2012·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Tomonori AoyamaDavid A Brenner
Dec 11, 2014·Annals of Clinical and Translational Neurology·Ying SiPeter H King
Feb 3, 2015·Annals of Clinical and Translational Neurology·Owen M PetersVladimir L Buchman
Jan 8, 2013·The International Journal of Neuroscience·Audrey DangoumauPatrick Vourc'h
Dec 20, 2011·Pharmacological Research : the Official Journal of the Italian Pharmacological Society·Mariarita GalbiatiAngelo Poletti
Dec 1, 2017·Acta Biochimica Et Biophysica Sinica·Risa Kashima, Akiko Hata
Oct 14, 2017·Journal of Neural Transmission·Marie-Victoire Guillot-Sestier, Terrence Town
Apr 8, 2014·American Journal of Physiology. Lung Cellular and Molecular Physiology·Trevor C StevensTroy Stevens
Oct 10, 2009·Journal of Neuropathology and Experimental Neurology·Yoshimi KinoshitaHirofumi Kusaka
Jul 28, 2019·Scientific Reports·Luke W BonhamUNKNOWN International FTD-Genomics Consortium (IFGC)
Jun 21, 2020·International Journal of Molecular Sciences·Mariarita GalbiatiAngelo Poletti
Aug 21, 2019·Ageing Research Reviews·Paramita ChaudhuriKarunakar Kar
Mar 22, 2021·Developmental Dynamics : an Official Publication of the American Association of Anatomists·Kathryn Russo, Kristi A Wharton

❮ Previous
Next ❯

Related Concepts

Related Feeds

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.

ALS: Genetics

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. Here is the latest research investigating genetic alterations in this genetically heterogeneous disorder.

ALS: Genetics

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. ALS is a genetically heterogeneous disorder with several causative genes. Here are the latest discoveries pertaining to the genetics of this disease.