Investigating which cells cause thickening of tissues in systemic sclerosis

Disease - Scleroderma

Lead applicant - Professor David Abraham

Organisation - University College London

Type of grant - Invited Research Award

Status of grant - Active

Amount of the original award - £249,434

Start date - 1 April 2018

Reference - 21810

Public Summary

What are the aims of this research?

Connective tissue contains a protein called collagen and having too much of this protein can cause parts of the body's tissues to thicken and stiffen. Systemic sclerosis (scleroderma) is an autoimmune condition in which there is an over-production of collagen. It can affect the skin, internal organs and blood vessels.

It is thought that a special type of cell, known as the fibroblast, is responsible for causing thickening of connective tissues. The aim of this work is to identify which type of fibroblasts play a role in this process, and to understand more about how this occurs.

Why is this research important?

In mice with systemic sclerosis, the researchers will identify the fibroblasts that are responsible for resulting in thickening of skin. They will look at a number of characteristics of these fibroblasts; for example, which genes they have and which type of chemicals they produce. They will also look at how they move, divide and produce the excessive collagen that causes the thickening of the skin.

The researchers will then investigate whether the fibroblasts that cause thickening of the skin in mice are the same fibroblasts that they've previously shown to be responsible for causing thickening of connective tissue in lungs of mice with systemic sclerosis. They will then compare these fibroblasts to fibroblasts from samples of patients with systemic sclerosis in order to make sure that any findings that they make in mice are also relevant to humans.

How will the findings benefit patients?

There is an urgent need to understand more about the processes involved in causing overproduction of collagen in systemic sclerosis. If this work is successful, it may identify which fibroblasts are responsible for causing thickening of skin, and it may also help improve the understanding of how they work. This could in turn allow to help identify new drugs to treat fibrosis.