Brain UK study ref: 26/004,

Lay summary,

Project status: Active

Investigation of mitochondrial dysfunction in Inclusion Body Myositis

Prof. Hector Chinoy, University of Manchester

Inclusion body myositis (IBM) is a slowly progressive muscle disease in adults over the age of 50. It causes increasing weakness in the thigh muscles and the muscles that bend the fingers, making everyday activities such as walking, climbing stairs, and swallowing progressively difficult. Over time, many people require mobility aids or a wheelchair, with a devastating and significant negative impact on daily life. IBM develops through two interconnected processes. First, the immune system mistakenly attacks healthy muscle cells, causing inflammation. At the same time, damaged muscle cells accumulate abnormal proteins and develop malfunctioning mitochondria (“power stations”). These processes reinforce each other, leading to gradual muscle wasting. Currently, there is no cure for IBM. The trigger for IBM is still not fully understood, which is where this project comes in. We will study existing IBM muscle biopsy samples using an advanced technique called spatial transcriptomics, which shows which genes are switched on or off in different areas of tissue, similar to reading error logs in a computer. This research aims to identify new treatment targets, an area where progress has been limited. We have Planned Patient and Public Involvement and Engagement sessions to inform the project throughout its timeframe.