The purpose of the described studies was to characterize the B-cell repertoire in WHIM subjects. Author information: (1)Metabolism, Infection and Immunity Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA. Perspect. Please enable it to take advantage of the complete set of features! Mitochondrial antiviral response. Role of mitochondrial dysfunction on rheumatic diseases. The event has been automatically approved because you are a manager for the calendar you submitted it to. Due to our privacy policy, only current members can send messages to people on ResearchGate. Our current understanding suggests that aerobic glycolysis is a hallmark of activated CD8+ T cells, while naive... Immunometabolism aims to define the role of intermediary metabolism in immune cell function, with bioenergetics and the mitochondria recently taking center stage. COVID-19 is an emerging, rapidly evolving situation. University students and faculty, institute members, and independent researchers, Technology or product developers, R&D specialists, and government or NGO employees in scientific roles, Health care professionals, including clinical researchers, Journalists, citizen scientists, or anyone interested in reading and discovering research. USA. Biol. Mitochondrial disorders as windows into an ancient organelle. -, Antico Arciuch V.G., Elguero M.E., Poderoso J.J., Carreras M.C. Indeed, oxidative stress has been demonstrated to be an underlying pathophysiologic process in various inborn errors of metabolism. peter.mcguire@nih.gov. Activation of innate immunity system during aging: NF-kB signaling is the molecular culprit of inflamm-aging. Once these mitochondrial components are released into the extracellular space, they can lead to the activation of innate and adaptive immune cells. Despite the vast amount of research dedicated to this subject, the relationship between aging mitochondria and immune function is largely absent from the literature. A 'read' is counted each time someone views a publication summary (such as the title, abstract, and list of authors), clicks on a figure, or views or downloads the full-text. Sci. Antioxid. Sorry. National Human Genome Research Institute (NHGRI) BG 10 RM 7N260 10 CENTER DR BETHESDA MD 20814 (301) 451-7716 mcguirepj@mail.nih.gov. doi: 10.1073/pnas.0703126104. This protein modification was detected by immunoblot and mass spectrometry (MS), and then comprehensively validated by chemical and biochemical methods. Disorders of mitochondrial oxidative phosphorylation (OXPHOS) represent some of the most common metabolic disorders presenting in childhood. Fueling Inflamm-Aging through Mitochondrial Dysfunction: Mechanisms and Molecular Targets. doi: 10.1101/cshperspect.a011403. USA.gov. Peter J. McGuire, M.D. 2008 Apr;7(2):83-105. doi: 10.1016/j.arr.2007.09.002. 2019 Oct 19;20(20):5183. doi: 10.3390/ijms20205183. My translational research program concentrates on immunometabolism from 2 perspectives: 1) the ability of the immune system to modulate mitochondrial metabolism in organs (e.g. Program of Microbiology and Immunology. Cross-Talk between Mitochondrial Dysfunction-Provoked Oxidative Stress and Aberrant Noncoding RNA Expression in the Pathogenesis and Pathophysiology of SLE. 2012;4:a011403. Shannon Kruk, R.N. 2014 Aug;56:154-63. doi: 10.1016/j.exger.2014.03.002. In addition to making energy, mitochondria are multifaceted, being involved in heat production, calcium storage, apoptosis, cell signaling, biosynthesis, and aging. peter.mcguire@nih.gov. Redox Signal. Get the latest public health information from CDC: https://www.coronavirus.gov. Chronic lymphocytic leukemia (CLL) arises from the clonal expansion of a CD5+ B lymphocyte that is thought not to undergo intraclonal diversification. Salminen A, Huuskonen J, Ojala J, Kauppinen A, Kaarniranta K, Suuronen T. Ageing Res Rev. Mitochondrial damage associated molecular patterns…, Mitochondrial damage associated molecular patterns (DAMPs). In this review, three main issues facing the aging immune system are discussed: (1) inflamm-aging; (2) susceptibility to infection and (3) declining T-cell function. Keywords: However, the role of ASS1 in these tissues remains to be defined. Epub 2007 Sep 20. (3)National Human Genome Research Institute, National Institutes of Health, 49 Convent Drive, 4A62, Bethesda, MD, 20892, USA. Only verified researchers can join ResearchGate and send messages to other members. Department of Genetics and Genomic Sciences, University of Texas Southwestern Medical Center, Universidade Federal do Rio Grande do Sul, Centre Hospitalier Régional Universitaire de Lille, Medical Center of the Johannes Gutenberg University, Division of Molecular Oncology and Immunology, National Human Genome Research Institute (NHGRI): National Institute of Health, Chemical individuality in T cells: A Garrodian view of immunometabolism, Mitochondrial disease disrupts hepatic allostasis and lowers the threshold for immune-mediated liver toxicity, Chronic kidney disease in propionic acidemia, AMPK Signaling Regulates Expression of Urea Cycle Enzymes in Response to Changes in Dietary Protein Intake, Mitochondrial Dysfunction and the Aging Immune System, Macrophage derived TNFα promotes hepatic reprogramming to Warburg-like metabolism, Vulnerability of pediatric patients with mitochondrial disease to vaccine preventable diseases. 2019 Jul;165:181-195. doi: 10.1016/j.bcp.2019.03.008. Since cytochrome c oxidase (COX) is a... Metabolic decompensation in inborn errors of metabolism (IEM) is characterized by a rapid deterioration in metabolic status leading to life-threatening biochemical perturbations (e.g. At rest, naïve T-cells primarily use…, NLM peter.mcguire@nih.gov. 2007;104:14418–14423. The severe metabolic instability and increased mortality experienced by many affecteds, especially those with mut0 MMA, has led to ce... Nutritional management of acute metabolic decompensation in amino acid inborn errors of metabolism (AA IEM) aims to restore nitrogen balance. Sorry, you need to be a researcher to join ResearchGate. Biology (Basel). Using VHDJH cDNA single strand conformation polymorphism analyses, we detected intraclonal mobility variants in 11 of 18 CLL cases. National Human Genome Research Institute (NHGRI) National Institutes of Health Building 10 Room 5-1485A 10 Center Drive Bethesda, Maryland 20892 (301) 496-0133 skruk@mail.nih… The recognition of viral nucleic acids involves mitochondria and intact membrane potential (ψ. T-cell activation and differentiation involved metabolic reprogramming. We con... Free radical formation resulting in oxidative stress is a hallmark of mitochondrial dysfunction. doi: 10.1038/nature11707. See this image and copyright information in PMC. Epub 2014 Mar 7. The lifetime risk of breast cancer may approach 80-90% in women who have germline mutations of either of two genes, BRCA1 or BRCA2. Picca A, Lezza AMS, Leeuwenburgh C, Pesce V, Calvani R, Landi F, Bernabei R, Marzetti E. Int J Mol Sci. While nutritional recommendations have been published, they have never been rigorously evaluated. While patients may be asymptomatic during the prenatal or immediate postnatal periods, patients... Ornithine transcarbamylase deficiency (OTCD, OMIM# 311250) is an inherited X-linked urea cycle disorder that is characterized by hyperammonemia and orotic aciduria. -, Vafai S.B., Mootha V.K. Despite judicious monitoring and care, patients with fatty acid oxidation disorders may experience metabolic decompensation due to infection which may result in rhabdomyolysis, cardiomyopathy, hypoglycemia and liver dysfunction and failure. Join ResearchGate to find the people and research you need to help your work. 2017 Apr 28;18(5):933. doi: 10.3390/ijms18050933. Dr. McGuire uses animal and disease models to understand the interplay between mitochondrial metabolism and the immune system. | A prospective analysis of interim HA e... Citrullinemia type 1 (CTLN1) is an autosomal recessive disorder of metabolism caused by a deficiency of argininosuccinate synthetase. adaptive immunity; aging; immunosenescence; inflammation; innate immunity; mitochondria. 12:20pm, Storrs CampusWilfred B. sequence analyses indicated that these variants represented un... We aimed to find out the proportion of breast cancers in Ashkenazi Jewish women attributable to the frameshift mutation at position 185 involving the deletion of adenine and guanine (185delAG) in the breast cancer gene BRCA1. Multidisciplinary Digital Publishing Institute (MDPI). In a model system previously published by our group, we noted... Medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency (MCADD) is an autosomal recessive inherited fatty acid oxidation disorder. Epub 2019 Mar 9. Mitochondria are ancient organelles that have co-evolved with their cellular hosts, developing a mutually beneficial arrangement. Tsai CY, Shen CY, Liao HT, Li KJ, Lee HT, Lu CS, Wu CH, Kuo YM, Hsieh SC, Yu CL. Dr. McGuire is board certified in pediatrics, clinical genetics and biochemical genetics. By binding their cognate ligands or by direct interaction (i.e., reactive oxygen species, ROS), intracellular signaling pathways such as NFkB and the NLRP3 inflammasome become activated resulting in a proinflammatory response. It will appear in the events list if you refresh the page or navigate to the calendar it was submitted to. The recognition of viral nucleic acids involves mitochondria and intact…, T-cell activation and differentiation involved…, T-cell activation and differentiation involved metabolic reprogramming. Molecular and Cellular Bases of Immunosenescence, Inflammation, and Cardiovascular Complications Mimicking "Inflammaging" in Patients with Systemic Lupus Erythematosus. B Biol. DAMPs derived from mitochondrial components may be released during cellular injury, apoptosis or necrosis. Biochem Pharmacol. National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA Correspondence Peter J. McGuire, MS, MBBCh, NHGRI/NIH, 10 Center Drive, Room 7N260A, Bethesda, MD 20892, USA. eCollection 2020. Philos. Young Building, Room 100, Special Seminar hosted by UConn Department of Animal Science and Department of Nutritional Sciences, Dr. Peter McGuire We hypothesized that activation... Restimulation-induced cell death (RICD) regulates immune responses by restraining effector T cell expansion and limiting nonspecific damage to the host. The Urea Cycle Disorders Consortium (UCDC) is a member of the NIH funded Rare Diseases Clinical Research Network and is performing a longitudinal study of 8 urea cycle disorders (UCDs) with initial enrollment beginning in 2006.
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