biostudies-literatureChen JNational Institute of Neurological Disorders and StrokeHong Kong Research Grants Council HKSARBrightFocus FoundationNINDS NIH HHS60https://www.ebi.ac.uk/biostudies/studies/S-EPMC4991002biostudies-literatureUnknown11(1)<h4>Background</h4>Our previous studies of Alzheimer's disease (AD) suggested that glutamine broadly improves cellular readiness to respond to stress and acts as a neuroprotectant both in vitro and in AD mouse models. We now expand our studies to a second neurodegenerative disease, ataxia-telangiectasia (A-T). Unlike AD, where clinically significant cognitive decline does not typically occur before age 65, A-T symptoms appear in early childhood and are caused exclusively by mutations in the ATM (A-T mutated) gene.<h4>Results</h4>Genetically ATM-deficient mice and wild type littermates were maintained with or without 4 % glutamine in their drinking water for several weeks. In ATM mutants, glutamine supplementation restored serum glutamine and glucose levels and reduced body weight loss. LosMolecular neurodegenerationThe impact of glutamine supplementation on the symptoms of ataxia-telangiectasia: a preclinical assessment.PMC4991002GRF660813R01 NS0710221R01NS071022AD2012101Hart RPLi JChen JZhang YChow HPlummer MRChen YVail GHerrup KLouie LfalseThe impact of glutamine supplementation on the symptoms of ataxia-telangiectasia: a preclinical assessment.<h4>Background</h4>Our previous studies of Alzheimer's disease (AD) suggested that glutamine broadly improves cellular readiness to respond to stress and acts as a neuroprotectant both in vitro and in AD mouse models. We now expand our studies to a second neurodegenerative disease, ataxia-telangiectasia (A-T). Unlike AD, where clinically significant cognitive decline does not typically occur before age 65, A-T symptoms appear in early childhood and are caused exclusively by mutations in the ATM (A-T mutated) gene.<h4>Results</h4>Genetically ATM-deficient mice and wild type littermates were maintained with or without 4 % glutamine in their drinking water for several weeks. In ATM mutants, glutamine supplementation restored serum glutamine and glucose levels and reduced body weight loss. Los2016-01-01T00:00:00Z2016 Aug2025-04-26T15:55:59.032Z2019-06-06T16:14:21ZS-EPMC49910022753849610.1186/s13024-016-0127-y