دورية أكاديمية
Imaging brain glucose metabolism in vivo reveals propionate as a major anaplerotic substrate in pyruvate dehydrogenase deficiency.
| العنوان: | Imaging brain glucose metabolism in vivo reveals propionate as a major anaplerotic substrate in pyruvate dehydrogenase deficiency. |
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| المؤلفون: | Marin-Valencia I; The Abimael Laboratory of Neurometabolism, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA; Laboratory of Developmental Neurobiology, The Rockefeller University, New York, NY, USA. Electronic address: isaac.marin-valencia@mssm.edu., Kocabas A; The Abimael Laboratory of Neurometabolism, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA., Rodriguez-Navas C; The Abimael Laboratory of Neurometabolism, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA., Miloushev VZ; Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, USA., González-Rodríguez M; The Abimael Laboratory of Neurometabolism, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA., Lees H; Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, USA., Henry KE; Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, USA., Vaynshteyn J; The Abimael Laboratory of Neurometabolism, Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA., Longo V; Small Animal Imaging Core Facility, Memorial Sloan Kettering Cancer Center, New York, NY, USA., Deh K; Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, USA., Eskandari R; Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, USA; Department of Biochemistry, Albert Einstein College of Medicine, Bronx, NY, USA., Mamakhanyan A; Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, USA., Berishaj M; Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, USA., Keshari KR; Radiology, Memorial Sloan Kettering Cancer Center, New York, NY, USA; Molecular Pharmacology Program, Memorial Sloan Kettering Cancer Center, New York, NY, USA; Weill Cornell Medical College, New York, NY, USA. Electronic address: rahimikk@mskcc.org. |
| المصدر: | Cell metabolism [Cell Metab] 2024 Jun 04; Vol. 36 (6), pp. 1394-1410.e12. |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Cell Press Country of Publication: United States NLM ID: 101233170 Publication Model: Print Cited Medium: Internet ISSN: 1932-7420 (Electronic) Linking ISSN: 15504131 NLM ISO Abbreviation: Cell Metab Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Cambridge, Mass. : Cell Press, c2005- |
| مواضيع طبية MeSH: | Pyruvate Dehydrogenase Complex Deficiency Disease*/metabolism , Brain*/metabolism , Brain*/diagnostic imaging , Glucose*/metabolism , Propionates*/metabolism, Animals ; Mice ; Diet, Ketogenic ; Mice, Inbred C57BL ; Disease Models, Animal ; Male ; Glycolysis |
| مستخلص: | A vexing problem in mitochondrial medicine is our limited capacity to evaluate the extent of brain disease in vivo. This limitation has hindered our understanding of the mechanisms that underlie the imaging phenotype in the brain of patients with mitochondrial diseases and our capacity to identify new biomarkers and therapeutic targets. Using comprehensive imaging, we analyzed the metabolic network that drives the brain structural and metabolic features of a mouse model of pyruvate dehydrogenase deficiency (PDHD). As the disease progressed in this animal, in vivo brain glucose uptake and glycolysis increased. Propionate served as a major anaplerotic substrate, predominantly metabolized by glial cells. A combination of propionate and a ketogenic diet extended lifespan, improved neuropathology, and ameliorated motor deficits in these animals. Together, intermediary metabolism is quite distinct in the PDHD brain-it plays a key role in the imaging phenotype, and it may uncover new treatments for this condition. Competing Interests: Declaration of interests K.R.K. is a founder with equity interest of Atish Technologies, Inc. and a member of the scientific advisory boards of NVision Imaging Technologies, Imaginostics, and Mi2. K.R.K. holds patents related to imaging and modulation of cellular metabolism. I.M.-V., K.R.K., C.R.-N., M.G.R., and A.K. are in the process of a patent application for the use of propionate as a therapeutic agent for PDHD and other neurometabolic diseases. (Copyright © 2024 Elsevier Inc. All rights reserved.) |
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| معلومات مُعتمدة: | P30 CA008748 United States CA NCI NIH HHS; R01 CA237466 United States CA NCI NIH HHS; R01 CA252037 United States CA NCI NIH HHS; K08 NS110877 United States NS NINDS NIH HHS; S10 OD016432 United States OD NIH HHS; S10 OD028577 United States OD NIH HHS; R21 CA212958 United States CA NCI NIH HHS |
| فهرسة مساهمة: | Keywords: brain; glucose; imaging; ketogenic diet; metabolism; propionate; pyruvate; pyruvate dehydrogenase deficiency |
| المشرفين على المادة: | IY9XDZ35W2 (Glucose) 0 (Propionates) |
| تواريخ الأحداث: | Date Created: 20240605 Date Completed: 20240605 Latest Revision: 20240621 |
| رمز التحديث: | 20240621 |
| مُعرف محوري في PubMed: | PMC11187753 |
| DOI: | 10.1016/j.cmet.2024.05.002 |
| PMID: | 38838644 |
| قاعدة البيانات: | MEDLINE |
| تدمد: | 1932-7420 |
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| DOI: | 10.1016/j.cmet.2024.05.002 |