Study Guide-Exam - Spring 2017 Polayes

Carnitine and go with carnitine acyl transferase i

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carnitine and go with carnitine acyl transferase I and transfers fatty acid to carnitine and allows it to transport across both outer and inner membranes to go meet carnitine acyltransferase II and put it back on coenzyme A and repeat the process o Carnitine = Lys and Met o Deficiency in carnitine and its transferase Mild muscle cramping, weakness, death Tissue affected, muscle, kidney , heart Carnitine produced in the liver and kidneys Stored in skeletal muscles, heart, and brain Deficiency is caused by genetic disorders, high fat diet o This transport links the cytosolic pool of CoA with mitochondrial pool of CoA o Steps in degradation, can you take a lipid and break it down o What to do with unsaturated, mono-unsaturated, polyunsaturated fatty acids o Odd number fatty acids o Cobalamin enzymes Needs cobalt Catalyzes 3 types of reactions Intramolecular rearrangements, methylation, reduction of ribonucleotides to deoxyribonucleotides o Is biotin important? o It is important for odd numbered fatty acids o Biotin and B12 both help in the conversion of propionyl-CoA to succinyl-CoA so it can go into the citric acid cycle o Biotin (with the use of ATP) converts propionyl-CoA into D-Methylmalonyl-CoA o Coenzyme B12 converts L-Methylmalonyl-CoA into Succinyl-CoA o Coenzyme B12 is a complex cobalt-containing compound o Regulation o We don’t want fatty acid oxidation and fatty acid synthesis to happen at the same time o If we have lots of energy we do not want to be breaking down fat o So we are going to block the transport by malonyl-CoA
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o If we are making the substrates for doing synthesis we will turn off transport for breaking down fatty acids o Acetyl CoA Carboxylase (ACC) Enzyme that will make malonyl-CoA o Malonyl-CoA is going to block the transport Sample ?
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