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PMID:19816198
| Citation |
Snyder, AM, Wang, X, Patton, SM, Arosio, P, Levi, S, Earley, CJ, Allen, RP and Connor, JR (2009) Mitochondrial ferritin in the substantia nigra in restless legs syndrome. J. Neuropathol. Exp. Neurol. 68:1193-9 |
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| 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. |
| Links |
PubMed PMC3024883 Online version:10.1097/NEN.0b013e3181bdc44f |
| Keywords |
Aged; Aged, 80 and over; Cytosol/chemistry; Cytosol/metabolism; Female; Ferritins/biosynthesis; Ferritins/metabolism; Humans; Iron/deficiency; Iron/metabolism; Male; Middle Aged; Mitochondrial Proteins/biosynthesis; Mitochondrial Proteins/metabolism; Neurons/chemistry; Neurons/metabolism; Restless Legs Syndrome/metabolism; Restless Legs Syndrome/pathology; Substantia Nigra/chemistry; Substantia Nigra/metabolism; Substantia Nigra/pathology |
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