Neurofilament protein levels: quantitative analysis in essential tremor cerebellar cortex.

Neuroscience Letters
Elan D LouisPhyllis L Faust

Abstract

Essential tremor (ET) is among the most prevalent neurological diseases. A substantial increase in the number of Purkinje cell axonal swellings (torpedoes) has been identified in ET brains. We recently demonstrated that torpedoes in ET contain an over-accumulation of disorganized neurofilament (NF) proteins. This now raises the question whether NF protein composition and/or phosphorylation state in cerebellar tissue might differ between ET cases and controls. We used a Western blot analysis to compare the levels and phosphorylation state of NF proteins and α-internexin in cerebellar tissue from 47 ET cases versus 26 controls (2:1 ratio). Cases and controls did not differ with respect to the cerebellar levels of NF-light (NF-L), NF-medium (NF-M), NF-heavy (NF-H), or α-internexin. However, SMI-31 levels (i.e., phosphorylated NF-H) and SMI-32 levels (i.e., non-phosphorylated NF-H) were significantly higher in ET cases than controls (1.28±0.47 vs. 1.06±0.32, p=0.02; and 1.38±0.75 vs. 1.00±0.42, p=0.006). Whether the abnormal phosphorylation state that we observed is a cause of defective axonal transport and/or function of NFs in ET is not known. NF abnormalities have been demonstrated in several neurodegenerative diseases. Regardle...Continue Reading

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Citations

Apr 18, 2013·Current Neurology and Neuroscience Reports·Rodger J Elble
Sep 14, 2013·Brain : a Journal of Neurology·Rachel BabijElan D Louis
Apr 18, 2014·Current Neurology and Neuroscience Reports·Elan D Louis
Aug 9, 2015·Neurobiology of Disease·Eric BéliveauFrédéric Calon
Nov 5, 2014·Brain : a Journal of Neurology·Elan D LouisPhyllis L Faust
Oct 10, 2019·Glia·María Velasco-EstevezGraham K Sheridan
Jan 22, 2020·Nature Reviews. Neurology·Elan D Louis, Phyllis L Faust
Jun 20, 2020·Brain Structure & Function·Anne Marie WellsHelen Barbas
Sep 21, 2017·Experimental Brain Research·Thomas P WellingsRebecca Lim
Mar 30, 2021·Frontiers in Aging Neuroscience·Jennifer C MiguelElliott J Mufson
Sep 5, 2021·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Adryana ClementinoKumlesh K Dev

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