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Prof. F. Baas PhD

Position
Full Professor
Main activities
Patient care, Research
Specialisation
Human Genetics
Focus of research
  • Genetics
  • DNA diagnostics
  • Neurological disorders
Key publications
  • Schaffer Ashleigh E., Eggens Veerle R. C., Caglayan Ahmet Okay, Reuter Miriam S., Scott Eric, Coufal Nicole G., Silhavy Jennifer L., Xue Yuanchao, Kayserili Hulya, Yasuno Katsuhito, Rosti Rasim Ozgur, Abdellateef Mostafa, Caglar Caner, Kasher Paul R., Cazemier J. Leonie, Weterman Marian A., Cantagrel Vincent, Cai Na, Zweier Christiane, Altunoglu Umut, Satkin N. Bilge, Aktar Fesih, Tuysuz Beyhan, Yalcinkaya Cengiz, Caksen Huseyin, Bilguvar Kaya, Fu Xiang-Dong, Trotta Christopher R., Gabriel Stacey, Reis André, Gunel Murat, Baas Frank, Gleeson Joseph G. CLP1 founder mutation links tRNA splicing and maturation to cerebellar development and neurodegeneration Cell 2014;157 (3):651-663 [PubMed]
  • Weterman Marian A. J., Barth Peter G., van Spaendonck-Zwarts Karin Y., Aronica Eleonora, Poll-The Bwee-Tien, Brouwer Oebele F., van Tintelen J. Peter, Qahar Zohal, Bradley Edward J., de Wissel Marit, Salviati Leonardo, Angelini Corrado, van den Heuvel Lambertus, Thomasse Yolande E. M., Backx Ad P., Nürnberg Gudrun, Nürnberg Peter, Baas Frank Recessive MYL2 mutations cause infantile type I muscle fibre disease and cardiomyopathy Brain 2013;136 (1):282-293 [PubMed]
  • Weterman Marian A. J., Sorrentino Vincenzo, Kasher Paul R., Jakobs Marja E., van Engelen Baziel G. M., Fluiter Kees, de Wissel Marit B., Sizarov Aleksander, Nürnberg Gudrun, Nürnberg Peter, Zelcer Noam, Schelhaas H. Jurgen, Baas Frank A frameshift mutation in LRSAM1 is responsible for a dominant hereditary polyneuropathy Human molecular genetics 2012;21 (2):358-370 [PubMed]
  • Namavar Yasmin, Barth Peter G., Kasher Paul R., van Ruissen Fred, Brockmann Knut, Bernert Günther, Writzl Karin, Ventura Karen, Cheng Edith Y., Ferriero Donna M., Basel-Vanagaite Lina, Eggens Veerle R. C., Krägeloh-Mann Ingeborg, de Meirleir Linda, King Mary, Graham John M., von Moers Arpad, Knoers Nine, Sztriha Laszlo, Korinthenberg Rudolf, Dobyns William B., Baas Frank, Poll-The Bwee Tien, van der Aa Nathalie, Arts Willem F. M., Ades Lesley C., Bahi-Buisson Nadia, Battini Roberta, Bodamer Olaf, Boltshauser Eugen, Boycott Kym, Brueton Louise, Brussel Wim, Chandler K. E., Cowan Frances M., Crow Yanick, Debus Otfried, Demir Ercan, Hastanesi Gazi, Eason Jacqueline, Ferrie Colin D., Fisher Richard B., Foulds Nicola, Freeman Jeremy L., Gooskens Rob, Haeussler Martin, Hageman Gerard, Hammersen Gerhard, Horn Denise, Tijssen Marina A. J. Clinical, neuroradiological and genetic findings in pontocerebellar hypoplasia Brain 2011;134 (Part 1):143-156 [PubMed]
  • Bahia El Idrissi Nawal, Das Pranab K., Fluiter Kees, Rosa Patricia S., Vreijling Jeroen, Troost Dirk, Morgan B. Paul, Baas Frank, Ramaglia Valeria M. leprae components induce nerve damage by complement activation: identification of lipoarabinomannan as the dominant complement activator Acta neuropathologica 2015;129 (5):653-667 [PubMed]
All Publications
Curriculum Vitae

 

Professor Frank Baas (male) obtained his MD (1982) and his PhD (1986) at the University of Amsterdam. In 1996 he became head of the Neurogenetics laboratory and in 2002 professor of Neurogenetics at the AMC, University of Amsterdam. In 2006 he became Head of the Genome Analysis facility AMC. He is a board certified Clinical Molecular geneticist. Prof. Baas is a leading scientist in the field of neurogenetics. He has published over 250 peer reviewed papers and has patents on neuroregeneration and cancer therapy.

 

CURRENT POSITIONS
Visiting scientist Koch Institute, Massachusetts Institute of Technology
Head Genome Analysis facility AMC
Professor of Neurogenetics at AMC/ University of Amsterdam
Head of the Neurogenetics laboratory, AMC/ University of Amsterdam

President of the Dutch Society of Human Genetics


PREVIOUS POSITIONS
2010-2016 CSO Regenesance BV www.regenesance-pharma.com

1991-1996 Honorary Staff member, The Netherlands Cancer Institute
1991 Assistant Professor Department of Neurology, University of Amsterdam to specialise in human molecular (neuro) genetics
1989-1991 Visiting scientist at The Netherlands Cancer Institute, Division of Molecular Biology, establishing a research group on the regulation of gene expression and the identification of new mechanisms for multidrug resistance
1987-1988 Visiting scientist at the Massachusetts Institute of Technology, Cancer Center, Prof. Dr. D.E. Housman and Harvard Medical School, Dana Farber Cancer Institute, Prof. R.J. Arceci. Topic: Regulation and expression of mammalian P-glycoprotein genes

FELLOWSHIPS AND AWARDS
2009 Amsterdam Inventor Award 2nd price 2009
1986 Stipend from the Netherlands Organization for Research (NWO) to visit Prof. Dr. T. Maniatis’ Laboratory at Harvard University
1987-1992 Constantijn and Christiaan Huygens Fellowship form the Netherlands Organization for Research.
 

Research programmes

Prof. F. Baas PhD (Genome analysis / Neurogenetics)

Our mission is to identify the pathomechanisms of human genetic disease and develop new therapeutic strategies based on this knowledge.

The AMC laboratory of Genome analysis has a longstanding reputation in identification of disease genes. We use expression profiling and pathway analysis to dissect disease mechanisms. Genomics based drugs, like RNA antagonists are developed. To expedite this process, we also host the genetics core facility of the AMC.

Our research is focussed on neurodegenerative diseases, like peripheral neuropathies (Charcot-Marie-Tooth disease, CMT) and ponto-cerebellar hypoplasia (PCH), a fatal early onset neurodegenerative disease.

In this research we have identified many genes for neurogenetic diseases (CMT, ALS and PCH). Our strong link with the clinic allowed us to perform genotype-phenotype correlation. Genetic studies also showed that patients with severe neuropathies can have multiple mutations in different genes related to neuropathy. However, the contribution of additional mutations in monogenic disease causing genes is low, only a few cases show digenic inheritance. In our search for modifiers of peripheral neuropathies we identified the complement system as a major determinant for nerve regeneration. Both genetic defects of  and pharmacological intervention in activation of the complement system has a major impact of regeneration of the injured brain and nerve. Our current research is aimed at dissecting the riole of the innate immune system in neuroinflammation and its relation to disease progression. In this way we we made substantial progress in understanding the factors that influence recovery of the nervous system after trauma.

The PCH research line led to the identification of tRNA processing in brain development and degeneration. In 2008 we identified the TSEN genes as cause for PCH, this was followed by the identification of other RNA processing genes like CLP1 and EXOSC3. Our research is now directed to identify the mechanisms by which defects in (t)RNA processing lead to neurodegeneretion. To this end iPS and zebrafish models are developed.

Faculty
K. Fluiter PhD
A.L.M.A. ten Asbroek MEng PhD
F. van Ruissen MEng PhD
M.A.J. Weterman PhD

Postdocs
V. Ramaglia PhD

Others
M.E. Jakobs
S.B. Kenter
R. Zwart BSc

Current research funding
  • Prinses Beatrix Spierfonds