Prof. PhD D. Troost

Endowed Professor
Main activities
Education, Patient care
Focus of research

Our major goal is to understand the pathological mechansisms leading to neurodegeneration and cell death in neurodegenerative diseases, with special focus on amyotrophic lateral sclerosis (ALS).The current involvement is centred on molecular pathological studies of human ALS tissue and the clinical pathology of ALS variants and familial ALS. Professor Troost administers and manages the ALS Brain Tissue Bank of the AMC.

Key publications
  • Nachreiner T., Esser M., Tenten V., Troost D., Weis J., Krüttgen A. Novel splice variants of the amyotrophic lateral sclerosis-associated gene VAPB expressed in human tissues Biochemical and biophysical research communications 2010;394 (3):703-708 [PubMed]
  • Boer Karin, Troost Dirk, Timmermans Wendy, van Rijen Peter C., Spliet Wim G. M., Aronica Eleonora Pi3K-mTOR Signaling and AMOG Expression in Epilepsy-associated Glioneuronal Tumors Brain pathology (Zurich, Switzerland) 2010;20 (1):234-244 [PubMed]
  • Bleeker Fonnet E., Atai Nadia A., Lamba Simona, Jonker Ard, Rijkeboer Denise, Bosch Klazien S., Tigchelaar Wikky, Troost Dirk, Vandertop W. Peter, Bardelli Alberto, van Noorden Cornelis J. F. The prognostic IDH1( R132 ) mutation is associated with reduced NADP+-dependent IDH activity in glioblastoma Acta neuropathologica 2010;119 (4):487-494 [PubMed]
  • Schut E. S., Brouwer M. C., de Gans J., Florquin S., Troost D., van de Beek D. Delayed cerebral thrombosis after initial good recovery from pneumococcal meningitis Neurology 2009;73 (23):1988-1995 [PubMed]
  • Vance Caroline, Al-Chalabi Ammar, Ruddy Deborah, Smith Bradley N., Hu Xun, Sreedharan Jemeen, Siddique Teepu, Schelhaas H. Jurgen, Kusters Benno, Troost Dirk, Baas Frank, de Jong Vianney, Shaw Christopher E. Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2-21.3 Brain 2006;129 (Part 4):868-876 [PubMed]
All Publications
Curriculum Vitae

Professor Dirk Troost (male, 1951), obtained his MD in 1978 became board certified Neurologist in1983 and registered Neurologist and Neuropathologist in1984. He became consultant Neuropathologist in 1984 at the Academic Medical Center, University of Amsterdam. In 1992 he obtained his PhD and became in 1999 professor of Neuropathology and head of the department, University of Amsterdam Academic Medical Centre. In 1984 a brain tumor bank was started and in 1986 he started the ALS-Bank which presently contains well characterized tissue samples of over 225 ALS patients and control tissue. His main research interests are next to ALS, tumors of the central nervous system and infections. From 1999-2007 he served as secretary general to the European Confederation of Neuropathological societies (EURO-CNS) and from 2008-2010 as vice-president. He became president in 2010 and left the office in 2012. He developed and organizes the CME courses and the European consultancy examination (EFN) in Neuropathology (see

Research programmes

Prof. PhD D. Troost (Ubiquinated RNA binding proteins in ALS)

Our goal is to understand the pathological mechansisms leading to neurodegeneration and cell death in amyotrophic lateral sclerosis (ALS). The p62 protein (SQSTM1) discovered in 1986 appeared to be a so-called ubiquitin-binding scaffold protein that colocalizes with ubiquitinated protein aggregates in ALS. However, p62 is probably more than just a scaffold and involved in signalling, receptor internalization and protein turnover. In 2006 the RNA-binding protein TDP-43 was identified as pathological protein in the majority of fronto-temporal dementia (FTD) and most ALS cases. A second RNA-binding protein named FUS/TLS plays another important role in the pathogenesis of a subset of FTD and ALS. With antibodies to TDP-43, FUS and p62 we try to elucidate the borderline between normal and abnormal tissue in sporadic ALS, familial ALS, atypical cases of ALS and various forms of dementing neurodegenerative diseases not being classified. Since there is strong evidence that alterations in RNA processing might be a key event in the pathogenesis of these type of conditions we try to understand the relation between RNA-binding proteins and the development of inclusion body formation. This includes mainly neuropathological and immunohistochemical studies on human postmortem brain and spinal cord tissues.