Fig. 2From: Possible mechanisms for sensorineural hearing loss and deafness in patients with propionic acidemiaProposed pathophysiologic mechanism of long QT syndrome and sensoneurinal hearing loss in propionic acidemia. Accumulating metabolites such as propionic acid, methylcitrate and propionylcarnitine cause dysfunction of the KvLQT1/KCNE1 channel by acute blocking effects as well as reduced expression of the channel proteinBack to article page