Lecture 10 - Liver, Muscle, and Amino Acid Metabolism

Lecture 10 - Liver, Muscle, and Amino Acid Metabolism -...

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Metabolism of Fructose and Disorders - - Fructose is the other sugar form that we can from sucrose - Can be bad if we have large amounts - Fructose metabolism o Comes down another pathway in the liver (most of the fructose comes in this way) o Fructose fructokinase Fructose-1-P This enzyme is like that of hexokinase for glucose o Fructose-1-P Aldolase B Glyceraldehyde or Dihydroxyacetone P - The Dihydroxyacetone P can go through glycolysis - Glyceraldehyde can go through and make phosphoglycerides and - Def in these enzymes can result in Fructose mal-metabolism Lack of Fructokinase (Essential fructosuria) - Autosomal recessive - Benign (mild) conditions - Fructose accumulates in the urine (eventually will get metabolized Absence of Aldolase B [Hereditary fructose intolerance (“Fructose poisoning”)] - Autosomal recessive - Leads to intracellular trapping of fructose-1-P - Causes: hypoglycemia, vomiting, jaundice, hemorrhage, hepatomegaly, lacticacidemia, hyperuricemia - Also hepatic failure death - TX: rapid detection and removal of fructose and sucrose from diet! Normally we can deal with fructose intake unless we have these def. or if fructose is only source of diet! - Increase fructose ATP will drop if you deplete the Pi o ATP will go down ADP and AMP will go up o Turn on glycolysis o When this occurs the lactate concentrations get very high o This was a problem and can be life-threatening
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Lecture 10 – Liver, Muscle, and Amino Acid Metabolism Gluconeogenesis: Where does the energy come from? – requires 6ATP to make one glucose - Energy from FATTY ACIDS o When break FAs down to get acetyl CoA (2 units at time) o CoA will feed into the TCA and you get lots - For every acetyl CoA that goes into the TCA cycle two CO 2 ’s are lost before you can go to gluconeogenesis. o Thus there will never be a net synthesis of Glucose from Fatty Acids o There is no net gain in the carbons you are getting from the CO2 - Fatty Acids Provide the Energy for Gluconeogenesis, but they cannot provide the carbons for the net synthesis of Glucose - Carnitine Shuttles Fatty Acids into the
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Lecture 10 - Liver, Muscle, and Amino Acid Metabolism -...

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