Charles Hoppel, MD

Adjunct Professor



Send E-mail 


MD, St. Louis University School of Medicine, 1962
BS, Gonzaga University, 1959


Mitochondrial dysfunction occurs in many disorders, including obesity, insulin resistance, diabetes mellitus, metabolic syndrome, as well as genetic, cardiovascular, and neurological conditions. In mitochondrial diseases, skeletal muscle mitochondrial function, as a diagnostic tool helps to disclose defects in hard to diagnosis patients,. The study of mitochondrial physiology and dysfunction uncovered defects in aging cardiac myocardium, cardiac ischemia/reperfusion injury, and heart failure. For example: the occurrence of a specific defect in complex III in heart mitochondria localized between the myofibrils in aged rats was detected; this defect leads to enhance injury following ischemia/reperfusion in these elderly rats compared to adult animals. Treatment of the aging defect alleviated the increased injury in the aged rats. Mitochondrial metabolism produces metabolites that accumulate in the cell, plasma, and are excreted in the urine. Advanced techniques using ultra-high pressure liquid chromatography coupled to mass spectrometry lead to analysis of 75 acylcarnitines; these acylcarnitines, in tissues, plasma, and urine, can be determined in a chromatogram of just 14 minutes. This type of measurement provides assessment of metabolic issues to promote understanding of pathophysiology.


Sean Adams, Dmitry Grapov, Oliver Fiehn, Caitlin Campbell, Carol Chandler, Dustin Burnett, Elaine Souza, Gretchen Casazza, Nancy Keim, John Newman, Gary Hunter, Jose Fernandez, W.Timothy Garvey, Charles Hoppel, and Mary-Ellen Harper. Exercise plasma metabolomics and xenometabolomics in obese, sedentary, insulin-resistant women: impact of a fitness and weight loss intervention. Am J Physiol Endocrinol Metab. epress 2019 PMID:31526287

Hsiao CP, Chen MK, Veigl ML, Ellis R, Cooney M, Daly B, Hoppel C. Relationships between expression of BCS1L, mitochondrial bioenergetics, and fatigue among patients with prostate cancer. Cancer Manag Res. 11:6703-6717, 2019. PMID:31410061
Lai N, Kummitha C, Drumm M, Hoppel C. Alterations of skeletal muscle bioenergetics in a mouse with F508del mutation leading to a cystic fibrosis-like condition. Am J Physiol Endocrinol Metab. 317:E327-E336, 2019. PMID:31211618

Kim J, Lee K, Fujioka H, Tandler B, Hoppel CL. Cardiac mitochondrial structure and function in tafazzin-knockdown mice. Mitochondrion 43:53-62, 2018. PMID:30389594
Lai N, Kummitha C, Rosca MG, Fujioka H, Tandler B, Hoppel C. A comprehensive approach to skeletal muscle subsarcolemmal and interfibrillar mitochondrial bioenergetics. Acta Physiol (Oxf). 225:e13182, 2019. PMID:30168663

Hsiao CP, Hoppel C. Analyzing mitochondrial function in human peripheral blood mononuclear cells. Anal Biochem 549:12-20, 2018. PMID:29505781

Tandler B, Hoppel CL, Mears JA. Morphological Pathways of Mitochondrial Division. Antioxidants 7: pii: E30, 2018. PMID:29462856

Lesnefsky E.J., Chen Q., Tandler B. and Hoppel C.L., Mitochondrial Bioenergetics in the Heart. Mitochondrial disruption in cardiovascular diseases. In: Vasan R., Sawyer, D.(eds.) The Encyclopedia of Cardiovascular Research and Medicine, vol.[3], pp. 365-380, 2018. Oxford: Elsevier.
Lai, N, Kummith, C Hoppel C. Defects in skeletal muscle subsarcolemmal mitochondria in a non-obese model of Type 2 diabetes mellitus Plos ONE 12:e0183978. 2017. PMID:28850625

Sudeepa Bhattacharyya, Mohamed Ali, William H. Smith, Paul E. Minkler, Maria S. Stoll, Charles L. Hoppel, Sean H. Adams. Anesthesia and bariatric surgery gut preparation alter plasma acylcarnitines reflective of mitochondrial fat and branched-chain amino acid oxidation. Am J Physiol Endocrinol Metab 313:E690-E698, 2017. PMID:28830869

Minkler PE, Stoll MSK, Ingalls ST, and Hoppel CL. Selective, Accurate, and Precise Quantitation of Glutarylcarnitine in Human Urine from a Patient with Glutaric Acidemia Type I. J Appl Lab Med November, 2017 pp. 1-10. Supplemtent pp. 11-30.
Minkler PE, Stoll MSK, Ingalls ST, Hoppel CL. Selective and Accurate C5 Acylcarnitine Quantitation by UHPLCMS/MS: Distinguishing True Isovaleric Acidemia from Pivalate Derived Interference. J Chromatogr B Analyt Technol Biomed Life Sci. 1061-1062:128-133, 2017. PMID:28734160

Bedoyan JK, Yang SP, Ferdinandusse S, Jack RM, Miron A, Grahame G, DeBrosse SD, Hoppel CL, Kerr DS, Wanders RJ. Lethal neonatal case and review of primary short-chain enoyl-CoA hydratase (SCEH) deficiency associated with secondary lymphocyte pyruvate dehydrogenase complex (PDC) deficiency. Mol Genet Metab 120:342-349, 2017. PMID:28202214

Hoppel CL, Lesnefsky EJ, Chen Q, Tandler B. Mitochondrial Dysfunction in Cardiovascular Aging. Adv Exp Med Biol. 982:451-464, 2017. PMID:28551802

Minkler PE, Stoll MSK, Ingalls ST, Hoppel CL. Appropriate use of Mass Spectrometry in Clinical and Metabolic Research LC GC, Current Trends in Mass Spectrometry, 116:231-41, 2017. PMID:26458767

Minkler PE, Stoll MSK, Ingalls ST, Hoppel CL. Correcting False Positive Medium-Chain Acyl-CoA Dehydrogenase Deficiency Results from Newborn Screening; Synthesis, Purification, and Standardization of Branched-Chain C8 Acylcarnitines for use in their Selective and Accurate Absolute Quantitation by UHPLC-MS/MS. Mol Genet Metab. 120:363-369, 2017. PMID:28190699
^ top