Exploring Mechanisms of GRACILE: A Cancer-like Metabolic Change in Mitochondrial Disorders

A mitochondrial disease in Finland has a mechanism that resembles aging and cancer

In a study, it was found that newborns with a mitochondrial disorder show cancer-like changes to proliferating cell proliferation. This causes tissues to age prematurely. This finding represents a major step in the development of treatments for mitochondrial disease and understanding of the syndrome.

The GRACILE Syndrome, a mitochondrial disorder that is part of the Finnish inheritance diseases, has altered metabolism and cell proliferation that are similar to cancer cells. Similar mitochondrial diseases may be treated in the future by limiting excessive cellular proliferation. A study conducted by professor emerita Vineta Fellman and docent Jukka Kälijarvi at the Folkhalsan Center and University of Helsinki, and published in Nature Communications April 2023, shows that this is the case.

Mitochondria play a major role in the cellular metabolism of energy. Mutations of genes that are required for mitochondrial function can cause mitochondrial disease in humans. GRACILE Syndrome is caused by an abnormality in the respiratory system, which the mitochondria use to generate cell energy. The syndrome begins in the fetal stage and manifests after birth as severe liver, kidney and metabolic complications. The syndrome is usually fatal for newborns.

Source:
https://medicalxpress.com/news/2023-05-mechanism-resembling-aging-cancer-finnish.html

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