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[Abstract] Int Immunopharmacol

Glucosylceramide lipidosisGaucher disease

The present study was supported by grants from the National Natural Scientific Foundation of China (Grant Nos

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moderate preclinical evidence ALC plays a fundamental role in mitochondrial function by facilitating the transport of long-chain fatty acids across the inner mitochondrial membrane for -oxidation and ATP production.[7] Dietary supplementation with ALC counteracts age-related alterations of mitochondrial biogenesis, dynamics, and antioxidant defenses in the brain, increasing levels of PGC-1, PGC-1, NRF-1, and TFAMkey regulators of mitochondrial biogenesis.[8] ALC also counteracts the age-related increase of deleted mitochondrial DNA and restores mitochondrial content and function.[8][9] In hypoxic conditions, ALC-mediated neuroprotection is attributed to ERK1/2-Nrf2-regulated mitochondrial biosynthesis, effectively protecting hippocampal neurons from mitochondrial dysfunction, excitotoxicity, and neurodegeneration.[10] Combination Strategies for Mitochondrial Dysfunction: ALC 1,500-3,000 mg/day + CoQ10 100-300 mg/day (complementary electron transport chain support) + Alpha-lipoic acid 600 mg/day (complementary metabolic support) + B vitamins (cofactors for mitochondrial enzymes) 3