fig3

Organellar crosstalk as a potential therapeutic target for rare neurodegenerative diseases

Figure 3. Interactions between organelles for maintaining metabolic homeostasis under physiological conditions (Created in https://BioRender.com). Under healthy conditions, organelles work synergistically to maintain fatty acid and redox homeostasis within cells. Fatty acids are primarily synthesized in the endoplasmic reticulum (ER) by the elongation of very long-chain fatty acids proteins (ELOVL), with ELOVL1 and ELOVL6 being primarily responsible for elongating C16:0-coenzyme A (CoA) to C26:0-CoA. These long-chain and very long-chain fatty acids (VLCFAs; ≥ C22:0) are then utilized to support normal cellular functions or transported to peroxisomes and mitochondria for complete oxidation. Simultaneously, peroxisomes and mitochondria serve as major sites for reactive oxygen species (ROS) production but also possess antioxidant defense systems to mitigate oxidative stress. Accordingly, these organelle functions and interactions are essential for maintaining cellular metabolic homeostasis.

Rare Disease and Orphan Drugs Journal
ISSN 2771-2893 (Online)
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