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Projects / Programmes source: ARIS

Human cathepsin F: An unusual cysteine protease involved in neurodegeneration

Research activity

Code Science Field Subfield
1.05.00  Natural sciences and mathematics  Biochemistry and molecular biology   

Code Science Field
1.06  Natural Sciences  Biological sciences 
Keywords
Cathepsin F, cysteine cathepsins, inhihitors, neuronal ceroid lipofuscinosis, Kufs disease, neurodegeneration, structure, substrate specificity
Evaluation (rules)
source: COBISS
Points
2,365.79
A''
423.37
A'
961.32
A1/2
1,370.37
CI10
24,873
CImax
4,274
h10
57
A1
8.14
A3
1.44
Data for the last 5 years (citations for the last 10 years) on April 22, 2024; A3 for period 2018-2022
Data for ARIS tenders ( 04.04.2019 – Programme tender, archive )
Database Linked records Citations Pure citations Average pure citations
WoS  377  35,156  32,026  84.95 
Scopus  357  37,376  34,220  95.85 
Researchers (8)
no. Code Name and surname Research area Role Period No. of publicationsNo. of publications
1.  00449  PhD Iztok Dolenc  Biochemistry and molecular biology  Researcher  2020 - 2024  110 
2.  32503  PhD Katarina Karničar  Biotechnology  Researcher  2020 - 2024  24 
3.  55799  Tea Sinožić  Biochemistry and molecular biology  Junior researcher  2022 - 2024 
4.  14829  PhD Veronika Stoka  Biochemistry and molecular biology  Head  2020 - 2024  237 
5.  15969  Ivica Štefe  Biochemistry and molecular biology  Technical associate  2020 - 2024  36 
6.  07561  PhD Boris Turk  Biochemistry and molecular biology  Researcher  2020 - 2024  1,037 
7.  04988  PhD Dušan Turk  Biochemistry and molecular biology  Researcher  2020 - 2024  621 
8.  33762  PhD Robert Vidmar  Biochemistry and molecular biology  Researcher  2020 - 2024  148 
Organisations (2)
no. Code Research organisation City Registration number No. of publicationsNo. of publications
1.  0106  Jožef Stefan Institute  Ljubljana  5051606000  90,695 
2.  2990  Center of excellence for integrated approaches in chemistry and biology of proteins, Ljubljana  Ljubljana  3663388  724 
Abstract
Lysosomal cysteine cathepsins play key roles in various physiological and pathological processes, including aging, neurodegeneration and genetic diseases. Their disregulated activities result in numerous human diseases and consequently are promising drug targets. Among eleven human cysteine cathepsins, cathepsin F is one of the less studied and characterized proteases. The recent discovery of several mutations within the human cathepsin F gene was a significant breakthrough that has provided strong evidence that this enzyme is associated with Type B Kufs disease, an adult onset neuronal ceroid lipofuscinosis (CLN13), and other neurodegenerative disorders. The major focus of this research will be to study the effects of cathepsin F mutations on enzyme activity, processing of cathepsin F proenzyme and the biological role of its proregion containing a cystatin-like domain. In addition we will determine the structures of procathepsin F and mature cathepsin F in complex with its inhibitor(s) and specific substrates. The results will provide insight into the cathepsin F functional network at the molecular level, which serves as a general model system for NCLs and other neurodegenerative diseases. Our integrative approach will map out the topological and dynamic properties of the underlying biological networks. The project combines various fields of expertise such as biochemistry including proteomics, molecular and cell biology, X-ray crystallography and bioinformatics. The results of these studies will not only impact basic science but also provide important information contributing to the development of treatment strategies for Type B Kufs disease and other neurodegenerative diseases.
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