diff --git a/Glycogen-Storage-Disease-Type-VI.md b/Glycogen-Storage-Disease-Type-VI.md new file mode 100644 index 0000000..6aa64a5 --- /dev/null +++ b/Glycogen-Storage-Disease-Type-VI.md @@ -0,0 +1 @@ +
The precise prevalence of GSDVI is unknown. At least eleven circumstances have been reported in the medical literature, though this situation is more likely to be underdiagnosed because it can be difficult to detect in children with mild symptoms or adults with no symptoms. GSDVI is more widespread within the Old Older Mennonite population, with an estimated incidence of 1 in 1,000 people. Mutations in the PYGL gene cause GSDVI. The PYGL gene gives directions for making an enzyme referred to as liver glycogen phosphorylase. This enzyme is found only 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 simple sugar that is the primary energy source for most cells in the physique. PYGL gene mutations stop liver glycogen phosphorylase from breaking down glycogen successfully. Because liver cells cannot break down glycogen into glucose, people with GSDVI can have hypoglycemia and will use fats for vitality, leading to ketosis.

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