Possible mechanisms for sensorineural hearing loss and deafness in patients with propionic acidemia
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چکیده
منابع مشابه
Possible mechanisms for sensorineural hearing loss and deafness in patients with propionic acidemia
Propionic acidemia is an inborn error of metabolism caused by deficiency of the mitochondrial enzyme propionyl-CoA carboxylase. Sensorineural deafness and severe hearing loss have been described as long-term complications of this disease, however, the mechanism has not yet been elucidated. We have recently shown by patch clamping experiments and Western blots that acute and chronic effects of a...
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ژورنال
عنوان ژورنال: Orphanet Journal of Rare Diseases
سال: 2017
ISSN: 1750-1172
DOI: 10.1186/s13023-017-0585-5