Mitochondrial Ferritin in the Substantia Nigra in Restless Legs Syndrome

作者：Snyder Amanda M; Wang XinSheng; Patton Stephanie M; Arosio Paolo; Levi Sonia; Earley Christopher J; Allen Richard P; Connor James R^*

来源：Journal of Neuropathology and Experimental Neurology, 2009, 68(11): 1193-1199.

DOI：10.1097/nen.0b013e3181bdc44f

• Ferritin
• Iron
• Mitochondrion
• Neuron
• Restless leg syndrome

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.

• 出版日期2009-11