TY - JOUR
T1 - Untangling the origin and function of granulovacuolar degeneration bodies in neurodegenerative proteinopathies
AU - Wiersma, Vera I.
AU - Hoozemans, Jeroen J.M.
AU - Scheper, Wiep
PY - 2020/9/3
Y1 - 2020/9/3
N2 - In the brains of tauopathy patients, tau pathology coincides with the presence of granulovacuolar degeneration bodies (GVBs) both at the regional and cellular level. Recently, it was shown that intracellular tau pathology causes GVB formation in experimental models thus explaining the strong correlation between these neuropathological hallmarks in the human brain. These novel models of GVB formation provide opportunities for future research into GVB biology, but also urge reevaluation of previous post-mortem observations. Here, we review neuropathological data on GVBs in tauopathies and other neurodegenerative proteinopathies. We discuss the possibility that intracellular aggregates composed of proteins other than tau are also able to induce GVB formation. Furthermore, the potential mechanisms of GVB formation and the downstream functional implications hereof are outlined in view of the current available data. In addition, we provide guidelines for the identification of GVBs in tissue and cell models that will help to facilitate and streamline research towards the elucidation of the role of these enigmatic and understudied structures in neurodegeneration.
AB - In the brains of tauopathy patients, tau pathology coincides with the presence of granulovacuolar degeneration bodies (GVBs) both at the regional and cellular level. Recently, it was shown that intracellular tau pathology causes GVB formation in experimental models thus explaining the strong correlation between these neuropathological hallmarks in the human brain. These novel models of GVB formation provide opportunities for future research into GVB biology, but also urge reevaluation of previous post-mortem observations. Here, we review neuropathological data on GVBs in tauopathies and other neurodegenerative proteinopathies. We discuss the possibility that intracellular aggregates composed of proteins other than tau are also able to induce GVB formation. Furthermore, the potential mechanisms of GVB formation and the downstream functional implications hereof are outlined in view of the current available data. In addition, we provide guidelines for the identification of GVBs in tissue and cell models that will help to facilitate and streamline research towards the elucidation of the role of these enigmatic and understudied structures in neurodegeneration.
KW - Granulovacuolar degeneration bodies
KW - Lysosomes
KW - Neurodegenerative proteinopathies
KW - Tau pathology
UR - http://www.scopus.com/inward/record.url?scp=85090318885&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85090318885&partnerID=8YFLogxK
U2 - https://doi.org/10.1186/s40478-020-00996-5
DO - https://doi.org/10.1186/s40478-020-00996-5
M3 - Review article
C2 - 32883341
SN - 2051-5960
VL - 8
SP - 153
JO - Acta Neuropathologica Communications
JF - Acta Neuropathologica Communications
IS - 1
M1 - 153
ER -