Using patient fibroblasts to understand inclusion body myositis

A Spanish team compared the fibroblasts of 14 patients with inclusion body myositis (IBM) to 12 healthy controls.

The IBM fibroblasts were found to have:

• abnormal expression of 778 genes related to inflammationRéaction non spécifique suscitée par un agent pathogène., mitochondria, cell cycle regulation and metabolism; an expression pattern differing according to whether the disease is stabilised or in active phase;
• cytokine secretion three times higher than normal;
• reduced autophagy;
• and, in the mitochondria, a reduction in gene content and enzymeProtéine capable d’activer une réaction biochimique précise. activity, as well as an increase in oxidative stress and antioxidant defences.

These results confirm the presence of molecular disorders in fibroblasts from patients with IBM, which could constitute a promising cellular model of the disease.

Unravelling inclusion body myositis using a patient-derived fibroblast model. Cantó-Santos J, Valls-Roca L, Tobías E et al. J Cachexia Sarcopenia Muscle. 2023 Apr;14(2):964-977.