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| http://dx.doi.org/10.1136/jnnp.32.5.399 | DOI Listing |
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Coordination of the leucine-sensing Rag GTPase cycle by leucyl-tRNA synthetase in the mTORC1 signaling pathway.
Proc Natl Acad Sci U S A
June 2018
Department of Integrated OMICS for Biomedical Science, Yonsei University, Seoul 03722, South Korea;
A protein synthesis enzyme, leucyl-tRNA synthetase (LRS), serves as a leucine sensor for the mechanistic target of rapamycin complex 1 (mTORC1), which is a central effector for protein synthesis, metabolism, autophagy, and cell growth. However, its significance in mTORC1 signaling and cancer growth and its functional relationship with other suggested leucine signal mediators are not well-understood. Here we show the kinetics of the Rag GTPase cycle during leucine signaling and that LRS serves as an initiating "ON" switch via GTP hydrolysis of RagD that drives the entire Rag GTPase cycle, whereas Sestrin2 functions as an "OFF" switch by controlling GTP hydrolysis of RagB in the Rag GTPase-mTORC1 axis.
View Article and Find Full Text PDFNeuroprotective Effect of Creatine and Pyruvate on Enzyme Activities of Phosphoryl Transfer Network and Oxidative Stress Alterations Caused by Leucine Administration in Wistar Rats.
Neurotox Res
November 2017
Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Rua Ramiro Barcelos 2600, Porto Alegre, RS, 90035-003, Brazil.
Maple syrup urine disease is an autosomal metabolic disease caused by a deficiency of branched-chain α-keto acid dehydrogenase complex activity. In this disease occur the accumulation of the branched-chain amino acids leucine, isoleucine, and valine and their corresponding branched-chain α-keto acids in the tissues and body fluids. The affected patients may present psychomotor development delay and mental retardation.
View Article and Find Full Text PDFMetabolic adaptation of skeletal muscle to hyperammonemia drives the beneficial effects of l-leucine in cirrhosis.
J Hepatol
November 2016
Departments of Pathobiology, Gastroenterology and Hepatology, Cleveland Clinic, Cleveland OH.
Background & Aims: Increased skeletal muscle ammonia uptake with loss of muscle mass adversely affects clinical outcomes in cirrhosis. Hyperammonemia causes reduced protein synthesis and sarcopenia but the cellular responses to impaired proteostasis and molecular mechanism of l-leucine induced adaptation to ammonia induced stress were determined.
Methods: Response to activation of amino acid deficiency sensor, GCN2, in the skeletal muscle from cirrhotic patients and the portacaval anastomosis (PCA) rat were quantified.
The present report describes a mother and 2 children with leucine-induced hypoglycemia (LIH). Hypoglycemic episodes following high-protein meals first appeared at age 4-7 months. Leucine-stimulation tests triggered marked hyperinsulinism and hypoglycemia in the children and a milder but abnormal response in the mother.
View Article and Find Full Text PDFThe effects of 5 mM theophylline on L-phenylalanine, L-alanine, and L-leucine-induced insulin secretion were studied using the isolated perfused rat pancreas, varying the sequence of the stimuli. Theophylline in the absence or presence of 3 mM D-glucose caused a small amount of insulin release with a slow onset and a slight and steady increase. Theophylline had no effect on phenylalanine (5, 10, or 20 mM)-induced insulin release independent of the sequence of stimuli.
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