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J Neuropathol Exp Neurol. 2009 Nov;68(11):1193-9. doi: 10.1097/NEN.0b013e3181bdc44f.

Mitochondrial ferritin in the substantia nigra in restless legs syndrome.

Author information

1
Department of Neurosurgery, Pennsylvania State University College of Medicine, Hershey, Pennsylvania 17033, USA.

Abstract

Restless legs syndrome (RLS) is a neurological disorder that is thought to involve decreased iron availability in the brain. Iron is required for oxidative metabolism and plays a critical role in redox reactions in mitochondria. The recent discovery of mitochondrial ferritin (FtMt) provided the opportunity to identify a potential correlation between iron and mitochondrial function in RLS. Human substantia nigra (SN) and putamen autopsy samples from 8 RLS cases and 8 controls were analyzed. Mitochondrial ferritin levels in RLS SN tissue homogenate samples assessed by immunoblots had more FtMt than control samples (p < 0.01), whereas there were no significant differences in FtMt in the putamen samples. By immunohistochemistry, neuromelanin-containing neurons in the SN were the predominant cell type expressing FtMt. Staining in neurons in RLS samples was consistently greater than that in controls. Cytochrome c oxidase staining, which reflects numbers of mitochondria, showed a similar staining pattern to that of FtMt, whereas there was less immunostaining in the RLS cases for cytosolic H-ferritin. These results suggest that increased numbers of mitochondria in neurons in RLS and increased FtMt might contribute to insufficient cytosolic iron levels in RLS SN neurons; they are consistent with the hypothesis that energy insufficiency in these neurons may be involved in the pathogenesis of RLS.

PMID:
19816198
PMCID:
PMC3024883
DOI:
10.1097/NEN.0b013e3181bdc44f
[Indexed for MEDLINE]
Free PMC Article

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