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Old 01-20-2012
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Default glucose 6 phosphatase

guys as we know glucose 6 phosphatase deficiency results in Von Gierke's

Disease(A Glycogen Storage Disease).glucose 6 phosphatase is also involved in

gluconeogenesis, so in Von Gierke's ds gluconeogenesis will also be impaired ?
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Old 01-20-2012
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Yes it will also be impaired that is the reason of presence of severe fasting hypoglycemia in Von Gierkes Disease.
In Hers disease only Glycogen phosphorylase is depleteed from the Liver resulting in Mild Hypoglecymia as Gluconeogenesis is not affected in it.
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Old 01-20-2012
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Quote:
Originally Posted by step_enhancer View Post
guys as we know glucose 6 phosphatase deficiency results in Von Gierke's

Disease(A Glycogen Storage Disease).glucose 6 phosphatase is also involved in

gluconeogenesis, so in Von Gierke's ds gluconeogenesis will also be impaired ?
Glucose-6-phosphotase is a liver enzyme.once this enzyme is defecient its is defecient at the transcription level.
as in glycogenolysis this enzyme is used to make glucose so is in gluconeogenesis----both ways results in Hypoglycemia.
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