## Abstract Multiple system atrophy (MSA) is a clinicopathological entity distinct from idiopathic Parkinson's disease (PD) that is responsible for 5โ10% of cases of parkinsonism. Degeneration of nigral neurones is a feature of both diseases. A specific deficiency of mitochondrial complex I activit
Assessment of mitochondrial respiratory chain function in hyperphenylalaninaemia
โ Scribed by N. Kyprianou; E. Murphy; P. Lee; I. Hargreaves
- Publisher
- Springer
- Year
- 2009
- Tongue
- English
- Weight
- 155 KB
- Volume
- 32
- Category
- Article
- ISSN
- 0141-8955
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## Abstract Reports on mitochondrial respiratory chain (MRC) complex I (CI) dysfunction in the substantia nigra in Parkinson's disease (PD) support the oxidative stress hypothesis in the neuropathogenesis of PD. Studies in peripheral tissue have found variable decreased CI and occasionally other co
The aim of this study was to illustrate the difficulties in establishing a diagnosis of mitochondrial respiratory chain (MRC) disorders based on clinical grounds in combination with intermediate activities of the MRC enzyme complexes. We reviewed retrospectively all medical and laboratory records of