Designer Protein Project

Disease: Creutzfeldt-Jakob Disease (CJD)

Molecule that causes the disease: Prion protein
Change in DNA: codon 129 in the prion protein gene
Change in the protein: folded abnormally
How does that change in protein causes symptoms: The prion protein turns the healthy
protein into its unhealthy shape causing the proteins to clump up and which causes brain tissue
to deteriorate, leading to dementia or other mental symptoms that eventually lead to death
within one year of the diagnosis.
Who is impacted by the disease: The disease can be acquired sporadically, genetically or by
infection, but it mostly shows up in adults.
Possible treatments: Some treatments that patients receive when diagnosed with CreutzfeldtJakob Disease are ones that focus on making the patient feel more comfortable. Some
medicines that have been tested to cure CJD are steroids, antibiotics, and antiviral agents, but
all have been unsuccessful. The drugs clonazepam and sodium valproate may also be given to
an infected patient to help reduce irregular jerking movements.
Our Proposal: We have been given the challenging task of trying to come up with a cure for
Creutzfeldt-Jakob disease. We think that if we can figure out a way for the protein to be folded
the correct way then the disease will be abled to be stopped. One idea that we found on how to
cure CJD is by using the molecule luminescent conjugated polythiophenes (LCPs) to stop the
protein macromolecules from clumping together which will prevent the disease from occurring.

Sources:

Uli S. Herrmann1, Anne K. Schtz2,*, Hamid Shirani3,*, Danzhi Huang4, Dino Saban1,,
Mario Nuvolone1, Bei Li1, Boris Ballmer1, Andreas K. O. slund3,, Jeffrey J. Mason3,
Elisabeth Rushing1, Herbert Budka1, Sofie Nystrm3, Per Hammarstrm3, Anja
Bckmann5, Amedeo Caflisch4, Beat H. Meier2, K. Peter R. Nilsson3, Simone
Hornemann1, and Adriano Aguzzi1,1Institute of Neuropathology, University Hospital of
Zrich, University of Zrich, 8091 Zrich, Switzerland.2Physical Chemistry,
Eidgenssische Technische Hochschule (ETH) Zrich, 8093 Zrich,
Switzerland.3Department of Physics, Chemistry and Biology (IFM), Linkping University,
58183 Linkping, Sweden.4Department of Biochemistry, University of Zrich, 8057
Zrich, Switzerland.5Institut De Biologie Et Chimie Des Protines, UMR 5086
CNRS/Universit De Lyon 1, 69367 Lyon, France.Corresponding Author. E-mail:
Adriano.aguzzi{at}usz.ch (A.A.); Simone.hornemann{at}usz.ch (S.H.)* These Authors
Contributed Equally to This Work. Present Address: Department of Neurosurgery,
University Hospital Essen, 45147 Essen, Germany. Present Address: The Norwegian
University of Science and Technology, 7491 Trondheim, Norway., By Uli S. Herrmann,
Anne K. Schtz, Hamid Shirani, Danzhi Huang, Dino Saban, Mario Nuvolone, Bei Li,
Boris Ballmer, Andreas K. O. slund, Jeffrey J. Mason, Elisabeth Rushing, Herbert
Budka, Sofie Nystrm, Per Hammarstrm, Anja Bckmann, Amedeo Caflisch, Beat H.
Meier, K. Peter R. Nilsson, Simone Hornemann, Adriano Aguzzi, Science Translational
Medicine05 Aug 2015 : 299ra123, Focus, Research Article, and Podcast. "Structurebased Drug Design Identifies Polythiophenes as Antiprion Compounds." Science
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Publications, Harvard Health. "Creutzfeldt-Jakob Disease (CJD) - Harvard Health."

Harvard Health. N.p., n.d. Web. 07 Oct. 2016.
Pastore, Manuela, Steven S. Chin, Karen L. Bell, Zhiqian Dong, Qiwei Yang, Lizhu Yang,
Jue Yuan, Shu G. Chen, Pierluigi Gambetti, and Wen-Quan Zou. "Creutzfeldt-Jakob
Disease (CJD) with a Mutation at Codon 148 of Prion Protein Gene : Relationship with
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