Glycogen. Glycogen. Liver ~ 4 % 72 g Muscle ~ 1 % 245 g Extracellular glucose 0,1 % 10 g Adult - man 70 kg His liver 1,8 kg His muscle mass 35 kg Blood volume 10 l. Glycogen. Muscle: Glucose substrate for muscle glycolysis Liver: Glucose store, Export of hexose Blood sugar
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Adult - man 70 kg
His liver 1,8 kg
His muscle mass 35 kg
Blood volume 10 l
Cent. Nervous syst.
Inakt. Protein Kinase A
Aktive Protein Kinase A
Phosphorylase Kinase a
Glukose 1 P
Glucose 6-phosphatase: liver and kidney
Here is another liver with a pale, bulging surface. This time the liver is filled with glycogen in von Gierke's disease, the glycogen storage disease of children.
Severly enlarged liver, severe hypoglycemia, lactic acidosis, ketosis, hyperuricemia, hyperlipemia
When mannose 6-phosphate
tags are added to acid
maltase enzyme molecules,
the molecules stick to receptors
(docking sites) on the
muscle cells and are
carried deeper inside
the cells, where they're needed.
Without mannose 6-phosphate
tags, acid maltase
enzyme molecules can't
enter muscle cells from
ENZYME TREATMENT BENEFITS BABIES WITH POMPE'S DISEASE
Babies with a metabolic muscle disorder known as Pompe's disease,
or acid maltase deficiency, usually don't survive infancy because they lack a vital enzyme that normally breaks down glycogen in the heart and skeletal muscle cells.
Cardiac failure in infancy; liver, heart, muscle
Liver cirrhosis, death usually before 24 months
Phosphorylase stain: Absent
Muscle fibers stain yellowMyophosphorylase deficiency: McArdle's disease
Phosphorylase stain: Normal
more darkly than type I