Marcos Gouveia (Author), Hiroko Ikeshima-Kataoka (Author), Špela Zemljič Jokhadar (Author), Marko Vidak (Author), Biljana Stojković (Author), Jure Derganc (Author), Rui Travasso (Author), Mirjana Liović (Author)

Abstract

Keratins are one of the most abundant proteins in epithelial cells. They form a cytoskeletal filament network whose structural organization seriously conditions its function. Dynamic keratin particles and aggregates are often observed at the periphery of mutant keratinocytes related to the hereditary skin disorder epidermolysis bullosa simplex, which is due to mutations in keratins 5 and 14. To account for their emergence in mutant cells, we extended an existing mathematical model of keratin turnover in wild-type cells and developed a novel 2D phase-field model to predict the keratin distribution inside the cell. This model includes the turnover between soluble, particulate and filamentous keratin forms. We assumed that the mutation causes a slowdown in the assembly of an intermediate keratin phase into filaments, and demonstrated that this change is enough to account for the loss of keratin filaments in the cell's interior and the emergence of keratin particles at its periphery. The developed mathematical model is also particularly tailored to model the spatial distribution of keratins as the cell changes its shape.

Keywords

keratin;epidermoliza bullosa simplex;mutacija;epidermolysis bullosa simplex;mutation;

Data

Language: English
Year of publishing:
Typology: 1.01 - Original Scientific Article
Organization: UL MF - Faculty of Medicine
UDC: 61
COBISS: 34772697 Link will open in a new window
ISSN: 1422-0067
Views: 374
Downloads: 145
Average score: 0 (0 votes)
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Other data

Secondary language: Slovenian
Secondary keywords: keratin;epidermoliza bullosa simplex;mutacija;model faznega polja;enačba reakcije-difuzije-advekcije;
Type (COBISS): Article
Pages: str. 1-13
Volume: ǂVol. ǂ21
Issue: ǂiss. ǂ7
Chronology: 2020
DOI: 10.3390/ijms21072596
ID: 12717700