Branched-chain amino acids (BCAA) administration increases autophagy and the autophagic pathway in brain tissue of rats submitted to a Maple Syrup Urine Disease (MSUD) protocol

Fermo, Karoline Teixeira; Lemos, Isabela da Silva; Farias, Hémelin Resende; Rosso, Marina Peyrot; Effting, Pauline Souza; Leipnitz, Guilhian; Streck, Emílio L.

doi:10.1007/s11011-022-01109-y

Cited by 3 publications

(1 citation statement)

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“…Because activation of mTOR inhibits autophagy, which is essential for the removal of unneeded or damaged proteins and organelles, there is evidence that amino acids may contribute to cognitive decline and synaptic dysfunction at least in part via activation of mTOR (Meijer et al, 2015;Wang et al, 2022). In some other cases, e.g., in rats in the maple syrup urine disease model, BCAA administration has been reported to increase autophagy in brain tissue (Fermo et al, 2023). Regulation of mTOR by amino acids also plays an essential role in neuronal development, which was studied recently (Takei and Nawa, 2014).…”

Section: Maintenance Of Amino Acid Levels and Mitochondrial Homeostasismentioning

confidence: 99%

Role of amino acid metabolism in mitochondrial homeostasis

Hoppe

2023

Front. Cell Dev. Biol.

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Mitochondria are central hubs for energy production, metabolism and cellular signal transduction in eukaryotic cells. Maintenance of mitochondrial homeostasis is important for cellular function and survival. In particular, cellular metabolic state is in constant communication with mitochondrial homeostasis. One of the most important metabolic processes that provide energy in the cell is amino acid metabolism. Almost all of the 20 amino acids that serve as the building blocks of proteins are produced or degraded in the mitochondria. The synthesis of the amino acids aspartate and arginine depends on the activity of the respiratory chain, which is essential for cell proliferation. The degradation of branched-chain amino acids mainly occurs in the mitochondrial matrix, contributing to energy metabolism, mitochondrial biogenesis, as well as protein quality control in both mitochondria and cytosol. Dietary supplementation or restriction of amino acids in worms, flies and mice modulates lifespan and health, which has been associated with changes in mitochondrial biogenesis, antioxidant response, as well as the activity of tricarboxylic acid cycle and respiratory chain. Consequently, impaired amino acid metabolism has been associated with both primary mitochondrial diseases and diseases with mitochondrial dysfunction such as cancer. Here, we present recent observations on the crosstalk between amino acid metabolism and mitochondrial homeostasis, summarise the underlying molecular mechanisms to date, and discuss their role in cellular functions and organismal physiology.

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