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Biochemistry
Amino acid metabolism
Nitrogen and urea cycle
Citric acid cycle
Electron transport chain and oxidative phosphorylation
Gluconeogenesis
Glycogen metabolism
Glycolysis
Pentose phosphate pathway
Physiological changes during exercise
Cholesterol metabolism
Fatty acid oxidation
Fatty acid synthesis
Ketone body metabolism
Alkaptonuria
Cystinuria (NORD)
Hartnup disease
Homocystinuria
Maple syrup urine disease
Ornithine transcarbamylase deficiency
Phenylketonuria (NORD)
Essential fructosuria
Galactosemia
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Hereditary fructose intolerance
Lactose intolerance
Pyruvate dehydrogenase deficiency
Abetalipoproteinemia
Familial hypercholesterolemia
Hyperlipidemia
Hypertriglyceridemia
Glycogen storage disease type I
Glycogen storage disease type II (NORD)
Glycogen storage disease type III
Glycogen storage disease type IV
Glycogen storage disease type V
Mucopolysaccharide storage disease type 1 (Hurler syndrome) (NORD)
Mucopolysaccharide storage disease type 2 (Hunter syndrome) (NORD)
Fabry disease (NORD)
Gaucher disease (NORD)
Krabbe disease
Leukodystrophy
Metachromatic leukodystrophy (NORD)
Niemann-Pick disease type C
Niemann-Pick disease types A and B (NORD)
Tay-Sachs disease (NORD)
Cystinosis
Disorders of amino acid metabolism: Pathology review
Disorders of carbohydrate metabolism: Pathology review
Disorders of fatty acid metabolism: Pathology review
Dyslipidemias: Pathology review
Glycogen storage disorders: Pathology review
Lysosomal storage disorders: Pathology review
Glycogen metabolism
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glycogen regulation p. 83
glycogen metabolism p. 84
glycogen regulation and p. 84
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glycogen metabolism p. 84
insulin and p. 333
periodic acid-Schiff stain p. 123
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glycogen metabolism p. 84
glycogen metabolism p. 84
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glycogen regulation p. 71, 84
glycogen and p. 84
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glycogen regulation p. 84
Glucose is a 6-carbon molecule that’s used to make energy, in the form of adenosine triphosphate, or ATP.
Glucose is such an important energy source, that our body stores excess glucose in skeletal muscle cells and liver cells in the form of glycogen.
Glycogen is basically an enormous molecule or polymer, that’s made up of glucose molecules linked together by glycosidic bonds.
You can think of glycogen having a main chain, and there being multiple branches sprouting off of it.
These branches allow glycogen to be compact and capable of rapid addition and removal of glucose.
It’s a bit like growing a plum tree in a tiny house with a short ceiling.
The short ceiling limits the tree’s vertical growth, but the tree’s able to branch off, so that it can still grow and produce many plums in a tight space.
Now let’s say that you just wrapped up a delicious lunch - you had tacos! Glucose is absorbed from the intestine and our blood sugar goes up. The pancreas responds to high blood sugar by secreting insulin.
Insulin acts on glucose transporters on the cell membrane, which are called GLUTs - and makes them bring more glucose into all the cells in our body.
Inside the cell, an enzyme called hexokinase adds a phosphate group to it’s 6th carbon, creating glucose 6 phosphate.
Then, glucose-6-phosphate is broken down during glycolysis, making ATP as a byproduct.
Over time, ATP levels start to rise and that inhibits certain enzymes in glycolysis.
When that happens, the extra glucose-6 phosphate can be used to make glycogen. And that usually takes place in the liver and muscle cells.
There are four main steps in glycogen synthesis.
First is attaching a uridine diphosphate, or UDP molecule to glucose.
Second, is attaching the glucose part of the UDP-glucose molecule to a glycogen primer called glycogenin, forming a short linear glycogen chain, which serves as a primer.
Glucagon is a hormone that helps your body to break down glycogen (a type of sugar) in the liver to release glucose into the bloodstream. This can help to raise blood sugar levels when they are too low, like during fasting. Glucagon is produced by alpha cells of the islets of Langerhans in the pancreas.
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