POLG mutations causing ophthalmoplegia, sensorimotor polyneuropathy, ataxia, and deafness.

Mancuso, M; Filosto, M; Bellan, M; Liguori, R; Montagna, P; Baruzzi, A; DiMauro, S; Carelli, V · Neurology · 2004

case_report · Level V

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Abstract

The authors identified two novel heterozygous missense transitions in the gene for the mitochondrial polymerase gammaA subunit (POLG) in a family with an autosomal recessive syndrome comprising progressive external ophthalmoplegia (PEO), polyneuropathy, ataxia, sensorineural hearing loss, and affective disorders. These mutations were not detected in 120 healthy control subjects.

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