Association of low blood manganese concentrations with epilepsy

Carl, G. F.; Keen, Carl L.; Gallagher, Brian B.; Clegg, Michael S.; Littleton, W.; Flannery, David B.; Hurley, Lucille S.

doi:10.1212/wnl.36.12.1584

Cited by 58 publications

(22 citation statements)

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“…On the other hand, the conclusion by Carl et al (1986Carl et al ( , 1990) that differences in Mn2+ concentrations are related to genetically determined susceptibility to seizures are supported by our data indicating that glutamine synthetase activity is lower in brains of epileptic animals than in nonepileptic controls, paralleling an earlier observation (Carl et al, 1990) showing lower Mn2+ levels in brain of epilepsyprone animals. Although initially the seizure effect on Mn2+ concentrations appeared to explain the several observations of low Mn2+ blood levels in epileptic persons (Dupont and Harpur 1977;Papavasiliou et al, 1979;Dupont and Tanaka 1985;Carl et al, 1986;Akram et al, 1989) it now appears that even though that effect is real it is not the only relation between epilepsy and Mn2+. Our data appear to show that a relation exists between Mn2+ Table 4.…”

Section: Resultssupporting

confidence: 90%

Manganese and Epilepsy: Brain Glutamine Synthetase and Liver Arginase Activities in Genetically Epilepsy Prone and Chronically Seizured Rats

et al. 1993

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Low blood manganese (Mn2+) concentration is associated with epilepsy in humans and rats. The low Mn2+ concentration is attributed by some investigators to the seizure activity associated with the epilepsy, whereas others propose that the low Mn2+ concentration may be secondary to genetic mechanisms underlying the epilepsy. To begin to differentiate between these possibilities, Mn(2+)-binding enzymes of liver and brain (i.e., arginase and glutamine synthetase, respectively) were assayed in rats exposed to chronically induced seizures and in genetically epilepsy-prone rats (GEPRs). Chronic seizures caused a decrease in whole blood Mn2+ levels but did not affect brain Mn2+ concentrations. Arginase activity was increased in livers of rats with chronic seizure as compared with controls, but this difference was eliminated when Mn2+ was added to the assay. Brain glutamine synthetase activity was unaffected by chronic seizures, but the activity of this enzyme was significantly lower in GEPR brain than in control brain. Liver arginase activity tended to be lower in GEPRs, although the difference was not statistically significant. These data indicate that seizures affect liver arginase activity through changes in liver Mn2+ concentration, but GEPRs show abnormalities in Mn(2+)-dependent enzymes apparently independent of seizure activity.

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Section: Resultssupporting

confidence: 90%

Manganese and Epilepsy: Brain Glutamine Synthetase and Liver Arginase Activities in Genetically Epilepsy Prone and Chronically Seizured Rats

et al. 1993

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“…Malecki and Greger found that lipid peroxidation was greater in rat heart mitochondria in animals deficient in Mn compared to rats receiving adequate amounts of manganese, which inversely correlated with MnSOD activity [252]. Manganese deficiency has been identified in patients with sundry diseases, including epilepsy [253], diabetes mellitus [254], and patients receiving hemodialysis [255]. Future studies of how this manganese deficiency correlates with MnSOD activity and lipid peroxidation in these, and other, diseases may provide important insights into mechanisms of disease development.…”

Section: Effects Of Mnsod On Mitochondrial Integrity/functionmentioning

confidence: 99%

Manganese Superoxide Dismutase: Guardian of the Powerhouse

Holley

Bakthavatchalu

Velez-Roman

et al. 2011

IJMS

256

180

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The mitochondrion is vital for many metabolic pathways in the cell, contributing all or important constituent enzymes for diverse functions such as β-oxidation of fatty acids, the urea cycle, the citric acid cycle, and ATP synthesis. The mitochondrion is also a major site of reactive oxygen species (ROS) production in the cell. Aberrant production of mitochondrial ROS can have dramatic effects on cellular function, in part, due to oxidative modification of key metabolic proteins localized in the mitochondrion. The cell is equipped with myriad antioxidant enzyme systems to combat deleterious ROS production in mitochondria, with the mitochondrial antioxidant enzyme manganese superoxide dismutase (MnSOD) acting as the chief ROS scavenging enzyme in the cell. Factors that affect the expression and/or the activity of MnSOD, resulting in diminished antioxidant capacity of the cell, can have extraordinary consequences on the overall health of the cell by altering mitochondrial metabolic function, leading to the development and progression of numerous diseases. A better understanding of the mechanisms by which MnSOD protects cells from the harmful effects of overproduction of ROS, in particular, the effects of ROS on mitochondrial metabolic enzymes, may contribute to the development of novel treatments for various diseases in which ROS are an important component.

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“…Calcium is an important intracellular messenger and is involved in many neuronal processes including short-term memory (Dash et al., 2007). Manganese ions are toxic to neuronal cells at high concentrations (Perl and Olanow, 2007), and manganese deficiency is linked with epilepsy (Carl et al., 1986). CMIP was identified through a screen to identify large proteins expressed in the brain (Nagase et al., 2000) and encodes an adaptor protein thought to form part of the cytoskeleton (Grimbert et al., 2003).…”

Section: Main Textmentioning

confidence: 99%

Genetic Advances in the Study of Speech and Language Disorders

Newbury¹,

Monaco²

2010

Neuron

171

110

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SummaryDevelopmental speech and language disorders cover a wide range of childhood conditions with overlapping but heterogeneous phenotypes and underlying etiologies. This characteristic heterogeneity hinders accurate diagnosis, can complicate treatment strategies, and causes difficulties in the identification of causal factors. Nonetheless, over the last decade, genetic variants have been identified that may predispose certain individuals to different aspects of speech and language difficulties. In this review, we summarize advances in the genetic investigation of stuttering, speech-sound disorder (SSD), specific language impairment (SLI), and developmental verbal dyspraxia (DVD). We discuss how the identification and study of specific genes and pathways, including FOXP2, CNTNAP2, ATP2C2, CMIP, and lysosomal enzymes, may advance our understanding of the etiology of speech and language disorders and enable us to better understand the relationships between the different forms of impairment across the spectrum.

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Association of low blood manganese concentrations with epilepsy

Cited by 58 publications

References 0 publications

Manganese and Epilepsy: Brain Glutamine Synthetase and Liver Arginase Activities in Genetically Epilepsy Prone and Chronically Seizured Rats

Manganese and Epilepsy: Brain Glutamine Synthetase and Liver Arginase Activities in Genetically Epilepsy Prone and Chronically Seizured Rats

Manganese Superoxide Dismutase: Guardian of the Powerhouse

Genetic Advances in the Study of Speech and Language Disorders

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