The life and death of oligodendrocytes in vanishing white matter disease

Journal of Neuropathology and Experimental Neurology
Keith Van HarenJames M Powers

Abstract

Vanishing white matter disease (VWM) is a progressive cavitating disease of central white matter due to a deficiency of the translation initiation factor eIF2B. Oligodendrocytes appear to be numerically increased in some white matter areas, while decreased in others. We compared oligodendrocytes of cerebral, cerebellar, and pontine white matter from 5 VWM patients with those of age-matched controls by light microscopy and immunohistochemistry using antibodies to activated caspase-3, bak, bax, bcl-2, survivin, and Ki-67, as well as by the TUNEL technique. Oligodendrocytes were identified morphologically and quantified using an ocular grid. We observed statistically significant increases in their densities at all sites; Ki-67-labeled oligodendrocytes were identified in 2 of 5 patients. Apoptotic oligodendrocytes were documented in 3 of 5 patients, while bcl-2 and survivin labeling was observed in 2 of 5 and 2 of 2 patients, respectively. There was a trend toward an increase in apoptotic labeling of oligodendrocytes that was strongest in the cerebrum, the major locus of VWM, in the youngest and most severely affected patients. These data conclusively demonstrate increased oligodendrocytic densities in VWM; the increase is not an a...Continue Reading

References

Jun 1, 1995·Journal of Cellular Biochemistry·W Meikrantz, R Schlegel
Jun 1, 1994·Neuropathology and Applied Neurobiology·C S MorrisN Gregson
Mar 1, 1994·Annals of Neurology·R SchiffmannC R Kaneski
Apr 1, 1996·Neurochemical Research·W T Norton
Apr 1, 1996·Journal of Neuroscience Research·D A Muir, D A Compston
Jan 1, 1997·Annual Review of Neuroscience·D E Merry, S J Korsmeyer
Apr 1, 1997·Neurology·M S van der KnaapJ Valk
Apr 21, 1997·The Journal of Cell Biology·B D TrappW Macklin
Aug 26, 1998·Neurology·M S van der KnaapJ Valk
Nov 11, 1998·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·M FujimuraP H Chan
Aug 12, 1999·American Journal of Human Genetics·P A LeegwaterM S van der Knaap
Oct 26, 2000·Nature·J Yuan, B A Yankner
Mar 29, 2001·Journal of Neurocytology·Y Ridderstråle, P J Wistrand
Mar 19, 2002·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Allen M Gown, Mark C Willingham
Apr 10, 2002·Journal of Neuropathology and Experimental Neurology·Judith Grinspan
Jul 31, 2002·Brain Pathology·Manuel B Graeber, Linda B Moran
Oct 21, 2003·American Journal of Human Genetics·Marjo S van der KnaapJan C Pronk
Aug 1, 1993·Current Biology : CB·B A BarresM C Raff

❮ Previous
Next ❯

Citations

Feb 22, 2005·Nature Medicine·Jörg DietrichChristoph Pröschel
Sep 6, 2005·Journal of Neuropathology and Experimental Neurology·J Patrick van der VoornMarjo S van der Knaap
Jun 30, 2006·Mental Retardation and Developmental Disabilities Research Reviews·Jan C PronkMarjo S van der Knaap
Nov 8, 2008·Annals of the New York Academy of Sciences·Jeremy D SchmahmannChristopher M Filley
Jul 11, 2006·Journal of Neuropathology and Experimental Neurology·Barbara van KollenburgMarjo S van der Knaap
Apr 20, 2011·Der Nervenarzt·H Prange, T Weber
Nov 11, 2009·The Neurologist·Daniel J CostelloFlorian S Eichler
May 10, 2018·Brain Pathology·Marianna BugianiMarjo S van der Knaap
May 28, 2019·Neuromolecular Medicine·Melisa HerreroOrna Elroy-Stein
Jan 5, 2008·Acta Neuropathologica·Tanja KuhlmannWolfgang Brück
Mar 24, 2005·Annals of Neurology·Gerre VermeulenMarjo S van der Knaap
Jan 20, 2017·Frontiers in Molecular Neuroscience·Vera G VolpiMaurizio D'Antonio
Aug 7, 2019·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Gülay GüngörKürşad Aydın
Apr 16, 2020·Acta Neuropathologica Communications·Aurélien TrimouilleAnnie Laquerrière
Dec 13, 2019·Expert Review of Neurotherapeutics·Mahmoud Reza AshrafiAli Reza Tavasoli
Apr 7, 2007·Topics in Magnetic Resonance Imaging : TMRI·Kim M Cecil, Radmila Savcic Kos
Mar 17, 2009·Nature Neuroscience·Wensheng Lin, Brian Popko
Jun 24, 2017·Acta Neuropathologica·Marjo S van der Knaap, Marianna Bugiani
Mar 2, 2018·Scientific Reports·Lisanne E WisseTruus E M Abbink
Apr 25, 2018·Annals of Clinical and Translational Neurology·Melanie D KlokMarjo S van der Knaap
May 9, 2019·Physiological Reviews·Christine StadelmannMikael Simons
Dec 18, 2020·Frontiers in Cellular Neuroscience·Dieuwke Maria de Waard, Marianna Bugiani
Apr 25, 2006·Lancet Neurology·Marjo S van der KnaapGert C Scheper
Dec 8, 2021·Cell Death & Disease·Abigail H ClevelandTimothy R Gershon

❮ Previous
Next ❯

Related Concepts

Related Feeds

BCL-2 Family Proteins

BLC-2 family proteins are a group that share the same homologous BH domain. They play many different roles including pro-survival signals, mitochondria-mediated apoptosis and removal or damaged cells. They are often regulated by phosphorylation, affecting their catalytic activity. Here is the latest research on BCL-2 family proteins.

Apoptosis

Apoptosis is a specific process that leads to programmed cell death through the activation of an evolutionary conserved intracellular pathway leading to pathognomic cellular changes distinct from cellular necrosis

Related Papers

Journal of Neuropathology and Experimental Neurology
Barbara van KollenburgM S Van der Knaap
Journal of Neuropathology and Experimental Neurology
J Patrick van der VoornM S Van der Knaap
© 2022 Meta ULC. All rights reserved