Projects / Programmes
The EB adhesome as potential target for new therapy approaches
| Code |
Science |
Field |
Subfield |
| 3.07.00 |
Medical sciences |
Metabolic and hormonal disorders |
|
| Code |
Science |
Field |
| 3.02 |
Medical and Health Sciences |
Clinical medicine |
regenerative medicine, iPSC, 3D tissue models, adhesome, extracellular matrix, epidermolysis bullosa, mass spectrometry
Data for the last 5 years (citations for the last 10 years) on
April 24, 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 |
145 |
9,706 |
9,473 |
65.33 |
| Scopus |
140 |
11,851 |
11,582 |
82.73 |
Researchers (6)
Organisations (2)
Abstract
Epidermolysis bullosa (EB) is a disfiguring and debilitating hereditary skin fragility disorder consisting of four major subtypes. It is caused by mutations affecting 18 structural proteins of the skin. As these proteins have a unique role of providing structural support to cells and adhesion to the different skin layers, mutations impair their structure and function and result in fragile skin that blisters easily and severely, often causing open wounds. In dystrophic EB (DEB) mutations affect collagen VII, which helps anchor the top layer of skin. The adhesome is a term incorporating all structural and signaling proteins involved in cell adhesion. Very little is known about the adhesome in EB. We believe that by determining the EB adhesome and by comparing it to healthy cells, we will learn more about the function of the adhesome, the EB disease mechanisms involved and how this may be linked to squamous cell carcinoma development, which is the major cause of death in recessive DEB patients. To achieve this, we are basing this project on cutting edge biotechnology techniques, such as mass spectrometry, induced pluripotent stem cell technology and the generation and testing on our own in vitro 3D full skin equivalents for EB.
