Succinate in dystrophic white matter: a proton magnetic resonance spectroscopy finding characteristic for complex II deficiency
Open accessAbstract
A deficiency of succinate dehydrogenase is a rare cause of mitochondrial encephalomyopathy. Three patients, 2 sisters and 1 boy from an unrelated family, presented with symptoms and magnetic resonance imaging signs of leukoencephalopathy. Localized proton magnetic resonance spectroscopy indicated a prominent singlet at 2.40ppm in cerebral and cerebellar white matter not present in gray matter or basal ganglia. The signal was also elevated in cerebrospinal fluid and could be identified as originating from the two equivalent methylene groups of succinate. Subsequently, an isolated deficiency of complex II (succinate:ubiquinone oxidoreductase) was demonstrated in 2 patients in muscle and fibroblasts. One of the sisters died at the age of 18 months. Postmortem examination showed the neuropathological characteristics of Leigh syndrome. Her younger sister, now 12 months old, is also severely affected; the boy, now 6 years old, follows a milder, fluctuating clinical course. Magnetic resonance spectroscopy provides a characteristic pattern in succinate dehydrogenase deficiency.
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Under aerobic conditions, succinate concentrations in tissues and mitochondria are submillimolar, indicating that complex II does not operate at maximal velocity under standard conditions.
“The succinate concentration in different tissues and mitochondria is in the submillimolar range under aerobic conditions [22,72,73], indicating that complex II is not working at maximal velocity under 'standard conditions'.”
From Differential effects of complex II on mitochondrial ROS production and their relation to cardioprotective pre- and postconditioning (2013) · cites this paper as [73]
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References 37 · Cited by 81
Research works printed but not linked: 3
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- Pandit S, Lin A, Gahbauer H, et al. MR spectroscopy in neu- rocysticercosis. J Comput Assist Tomogr 2001;25:950 -952.
- Pinard JM, Marsac C, Barkaoui E, et al. Syndrome de Leigh et leukodystrophie par de ´ficit partiel en succinate de ´shydroge ´nase: re ´gression sous riboflavine. Arch Pediatr 1999;6:421-426.
- Authors
- Brockmann, K., Bjornstad, A., Dechent, P., Korenke, C. G., Smeitink, J., Trijbels, J. M. F., Athanassopoulos, S., Villagran, R., Skjeldal, O. H., Wilichowski, E., Frahm, J., & Hanefeld, F.
- Affiliations
- Universitätsmedizin Göttingen (DE)
- Max Planck Society (DE)
- Radboud University Nijmegen (NL)
- Oslo University Hospital (NO)
- Corresponding author
- Brockmann, Knut
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 2002-06-18
- Volume
- 52
- Issue
- 1
- Pages
- 38-46
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W1975806836
- Cited by
- 81
- References
- 37
- Citations by year
- 2025: 2 · 2024: 3 · 2023: 4 · 2022: 2 · 2021: 3 · 2019: 1 · 2018: 2 · 2017: 2 · 2016: 6 · 2015: 3 · 2014: 1 · 2013: 8 · 2012: 8
- FWCI
- 1.96
- Type
- journal article
- Language
- en
- Open access
- green
- hdl.handle.net
- Licence
- Locations
- Annals of Neurology (publishedVersion)
- PubMed (publishedVersion)
- MPG.PuRe (Max Planck Society) (submittedVersion, other-oa)
- Max Planck Institute for Plasma Physics (submittedVersion)
- GRO (submittedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Advanced MRI Techniques and Applications
- Keywords
- succinate dehydrogenase deficiency, mitochondrial encephalomyopathy, leukoencephalopathy, succinic acid, 1H NMR, Leigh syndrome, cerebrospinal fluid
- MeSH terms
- Brain, Child, Female, Humans, Infant, Male, Multienzyme Complexes, Magnetic Resonance Spectroscopy, Oxidoreductases, Pedigree, Protons, Succinate Dehydrogenase, Dementia, Vascular, Electron Transport Complex II
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