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The exact prevalence of GSDVI is unknown. A minimum of eleven circumstances have been reported in the medical literature, although this condition is prone to be underdiagnosed because it may be tough to detect in youngsters with mild signs or adults with no symptoms. GSDVI is extra frequent in the Old Older Mennonite inhabitants, with an estimated incidence of 1 in 1,000 people. Mutations in the PYGL gene trigger GSDVI. The PYGL gene gives directions for making an enzyme referred to as liver glycogen phosphorylase. This enzyme is discovered solely in liver cells, the place it breaks down glycogen into a sort of sugar referred to as glucose-1-phosphate. Additional steps convert glucose-1-phosphate into glucose, a easy sugar that's the primary vitality source for most cells in the physique. PYGL gene mutations prevent liver glycogen phosphorylase from breaking down glycogen effectively. Because liver cells cannot break down glycogen into glucose, individuals with GSDVI can have hypoglycemia and may use fats for power, resulting in ketosis.
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