Biomaterial Database

Mitochondrial dysfunction in progressive supranuclear palsy. 2002

David S Albers, and M Flint Beal

Department of Neurology and Neuroscience, Weill Medical College, Cornell University, Room A-503, 525 East 68th Street, New York, NY 10021, USA. daa2010@mail.med.cornell.edu

A progressive impairment of mitochondrial function has been suggested to play a critical role in the pathogenesis of several neurodegenerative diseases, including Parkinson's disease, Alzheimer's disease and Huntington's disease. Mitochondrial dysfunction can lead to number of deleterious consequences including impaired calcium buffering, generation of free radicals, activation of the mitochondrial permeability transition pore and secondary excitotoxicity. Progressive supranuclear palsy (PSP) is a rare neurological disorder characterized by the appearance of supranuclear gaze palsy and extrapyramidal symptoms [Arch. Neurol. 10 (1964) 333]. Although the etiological basis of PSP is unknown, compelling evidence from spectroscopy studies in PSP patients, biochemical studies in post-mortem PSP brain tissue and PSP cybrids has emerged that supports a contributory role of bio-energetic defects in the pathogenesis of PSP.

UI	MeSH Term	Description	Entries
D008928	Mitochondria	Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)	Mitochondrial Contraction,Mitochondrion,Contraction, Mitochondrial,Contractions, Mitochondrial,Mitochondrial Contractions
D010085	Oxidative Phosphorylation	Electron transfer through the cytochrome system liberating free energy which is transformed into high-energy phosphate bonds.	Phosphorylation, Oxidative,Oxidative Phosphorylations,Phosphorylations, Oxidative
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013494	Supranuclear Palsy, Progressive	A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7)	Ophthalmoplegia, Progressive Supranuclear,Progressive Supranuclear Ophthalmoplegia,Progressive Supranuclear Palsy 1,Steele-Richardson-Olszewski Syndrome,Palsy, Progressive Supranuclear,Progressive Supranuclear Palsy,Richardson's Syndrome,Steele-Richardson-Olszewski Disease,Supranuclear Palsy, Progressive, 1,Progressive Supranuclear Palsies,Richardson Syndrome,Steele Richardson Olszewski Disease,Steele Richardson Olszewski Syndrome,Supranuclear Ophthalmoplegia, Progressive,Supranuclear Palsies, Progressive