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tutorial9_ans - Tutorial 9 Biochem 300 Tutorial 9 Aug 9th...

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Tutorial 9 Biochem 300 – Tutorial 9 – Aug 9 th 2009 Topics: Regulation/Integration of metabolism
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1. [PS8] Predict the major consequence of each of the following enzymatic deficiencies: - Glucose 6-phosphatase in liver - Carnitine acyltransferase I in skeletal muscle - Thiolase in brain - Kinase in liver that synthesizes fructose- 2,6-bisphosphate
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Glucose 6-phosphatase? Removes the 6-P to make glucose Either the last step of gluconeogenesis or of glycogenolysis (glycogen breakdown) Phosphorylated glucose can’t be exported to the blood. Thus glucose would be trapped in the liver Low blood glucose and high liver glycogen content
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Carnitine acyltransferase I Long fatty acids can’t be transported to matrix and under go beta oxidation This will be a problem when acyl breakdown is needed (fasting and exercise) Muscle weakness, low blood glucose, and low ketone levels (high carnitine in blood too)
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Thiolase in brain During low blood glucose periods, thiolase makes two acetyl-CoA from acetoacetyl-CoA and CoA Acetoacetate would no longer be produced as a fuel in the brain
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Kinase in liver that makes F26P F26P will be made at a much lower level Normally, F26P strongly activates glycolysis through allosteric modulation of phosphofructokinase 1, therefore, glycolysis will be much slower than usual.
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2. [PS8] Predict the major consequence of each of the following mutations affecting glycogen utilization: - Loss of GTPase activity of the G-protein alpha subunit - Loss of the gene that encodes inhibitor 1 of protein phosphatase 1 - Loss of phosphodiesterase activity
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