Short telomeres and pathogenesis of pulmonary fibrosis

  • Research type

    Research Study

  • Full title

    Investigation of the mechanism and predisposition to pulmonary fibrosis in diseases associated with short telomeres

  • IRAS ID

    89835

  • Contact name

    Shreyans Gandhi

  • Contact email

    shreyans.gandhi@nhs.net

  • Sponsor organisation

    King's College Hospital NHS Foundation Trust

  • Research summary

    Telomeres are protective caps at the end of chromosomes which give stability to the cell during division. Telomeres shorten with each cell division and shortened telomeres are a marker of an ‘aged’ cell. When telomeres are critically short, the ‘aged’ cell stops dividing and undergoes ‘self-programmed death’. Pulmonary fibrosis (PF) is a serious condition of scarring of the lungs, and is associated with short telomeres. PF is a disease of old age, where the physiological processes involved in ageing causes the telomeres to shorten. There is no satisfactory treatment for PF and it results in progressive disability with difficulty in breathing, which is often fatal. Bone marrow failure syndromes (BMFS) have short telomeres, and it’s likely short telomeres cause attrition of the precursor blood making cells within the bone marrow. Interestingly, PF is the second leading cause of death in such BMFS, however the mechanism and reasons remain unknown. We wish to study mechanisms that cause scarring of lung when telomeres are short. We will study the telomere maintenance pathways, immune responses and wound healing properties of lung cells with shortened telomeres to test the hypothesis that either abnormal wound healing in the lung cells or aberrant immune responses in immune cells with short telomeres may cause the lung to scar, rather than heal after a noxious insult.

  • REC name

    London - Bromley Research Ethics Committee

  • REC reference

    13/LO/1865

  • Date of REC Opinion

    21 Jan 2014

  • REC opinion

    Further Information Favourable Opinion