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Healthy Flow Blood Supports You in Maintaining Healthy Blood Flow

The exact prevalence of GSDVI is unknown. At least eleven circumstances have been reported in the medical literature, although this condition is likely to be underdiagnosed as a result of it can be troublesome to detect in children with mild signs or adults with no symptoms. GSDVI is extra frequent within the Old Older Mennonite population, with an estimated incidence of 1 in 1,000 people. Mutations in the PYGL gene trigger GSDVI. The PYGL gene offers directions for making an enzyme known as liver glycogen phosphorylase. This enzyme is discovered only in liver cells, the place it breaks down glycogen into a kind of sugar referred to as glucose-1-phosphate. Additional steps convert glucose-1-phosphate into glucose, a simple sugar that's the primary power supply for most cells in the body. PYGL gene mutations prevent liver glycogen phosphorylase from breaking down glycogen successfully. Because liver cells can not break down glycogen into glucose, people with GSDVI can have hypoglycemia and may use fats for energy, resulting in ketosis.

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