Notes in Glycogen Metabolism and Related Disorders

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Published	08/15/2023	Glycogen is stored in {{c1::liver cells}} and {{c2::skeletal muscle cells}}
Published	08/15/2023	During the first step of creating glycogen, glucose is converted into {{c1::UDP-glucose}}
Published	08/15/2023	{{c1::Alpha 1,4 glycosidic bonds }}connect different UDP-glucose molecules together to form glycogen.
Published	08/15/2023	Branching in glycogen is formed through{{c1:: Alpha 1,6 glycosidic bonds}} between glucose molecules.
Published	08/15/2023	{{c1::Glucagon}} directs liver cells to metabolize glycogen, while {{c2::epinephrine}} directs skeletal muscle cells to metabolize glycogen.
Published	08/15/2023	Glycogen is broken off into individual molecules of {{c1::glucose-1-phosphate}} which is then converted into {{c2::glucose-6-phosphate}}.
Published	08/15/2023	In the liver during glycogen breakdown, glucose-6-phosphate is converted into {{c1::free glucose}}
Published	08/15/2023	In the skeletal muscle during glycogen breakdown, glucose-6-phosphate is {{c1::inserted directly into glycolysis pathway.}}
Published	08/15/2023	{{c1::Glycogen synthase}} is active in a dephosphorylated state, while {{c2::glycogen phosphorylase}} is active in a phosphorylated state
Published	08/15/2023	{{c1::Glycogen storage disease type 1}} is also known as {{c2::Von-Gierke’s Disease}}.
Published	08/15/2023	{{c1::Glycogen storage disease type 1}} is caused by a mutation in the {{c2::glucose-6-phosphatase}} gene on {{c3::chromosome 17}}.
Published	08/15/2023	Glycogen storage disease {{c1::type 1 }}prevents the breakdown of glycogen into glucose in {{c2::liver cells}}.
Published	08/15/2023	What are the two ways that patients with glycogen storage disease type 1 can break down glucose-6-phosphate?
Published	08/15/2023	Glycogen storage disease type 1 has an inheritance pattern of {{c1::autosomal recessive}}
Published	08/15/2023	How is glycogen storage disease type 1 diagnosed?
Published	08/15/2023	How is glycogen storage disease type 1 treated?
Published	08/15/2023	{{c1::Glycogen storage disease type 2}} is also known as {{c2::Pompe disease}}
Published	08/15/2023	{{c1::Pompe disease}} is caused by a mutation of the {{c2::GAA gene}} that produces {{c3::acid alpha glucosidase}}
Published	08/15/2023	In patients with Pompe disease, glycogen tends to accumulate in the {{c1::lysosomes}} and damage muscles and other cell types
Published	08/15/2023	Glycogen storage disease type 2 has an inheritance pattern of {{c1::autosomal recessive}}
Published	08/15/2023	What is the main difference between early onset and late onset Pompe disease?
Published	08/15/2023	How is Pompe disease typically diagnosed?
Published	08/15/2023	How is Pompe disease typically treated?
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