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Description

V Farewell

Genetic testing in motor neurone disease

A c opax yca a

R323-01

SDH and fumarate hydratase (FH) catalyze sequential steps in the TCA cycle, and their inactivation results in accumulation of succinate and fumarate, respectively, both of which are structural analogues of 2OG (succinate is also a product of the 2OG-dependent dioxygenase-catalyzed reactions) and have been shown to inhibit the PHD enzymes at pathophysiological concentrations