BCH210H1 Lecture Notes - Lecture 36: Carnitine Palmitoyltransferase Ii, Glycogen Storage Disease, Aldehyde Dehydrogenase
25 May 2018
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Lecture 36: Case Studies
Alcohol Metabolism in humans
You’e just suied the BCHH fial ea ad deide to go dikig ith ou fied. Afte doig a too a
gin and tonics, you both return home tired and hungry. Your friend convinces you that you should apply your
ioheist koledge ad hek ou lood suga eause suel that’s h ou hae the uhies ad is oied
ou ight pass out. Ho a ou eplai ioheiall hat’s goig o i ou od?
Alcohol ad metabolic acidosis
• Ethanol is metabolized to acetate by alcohol dehydrogenase
and aldehyde dehydrogenase
Whats going on biochemically following alcohol consumption?
• Hormone
• Energy molecules
• Active pathways
• Inactive pathways
Case study – rhabdomyolysis
• Skeletal muscle damage leading to leakage of cellular components into the blood or urine
• Myoglobinuria – tea coloured urine, quantified by heme detection
• Also results in increased serum creatine kinase
• Can occur due to direct muscle trauma (crush syndrome) or excessive activity, temperature extremes, ischemia,
drugs or toxins, infection, metabolic disorders or genetically inherited disorders
McArdle Disease
• Glycogen storage disease, type V
• Autosomal recessive mutations in the PYGM
gene encoding for myophosphorylase
• Fatigue and muscle weakness
• Enzyme activity
• Second wind phenomena
• Treatment
• Complications
Carnitine Palmitoyltransferase II
• Fatty acyl chains are conjugate to carnitine for import into the mitochondria
• Beta oxidation can then produce ATP
• CPT-II is specific for 16 carbon chains
• Deficiency
- What symptoms
- Enzyme activity
- Conditions leading to symptoms
- Potential treatment
find more resources at oneclass.com
find more resources at oneclass.com
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BCH210H1 Full Course Notes
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