Term
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Definition
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Term
How does pyruvate get into mitochondrial matrix? |
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Definition
| pyruvate translocase (antiport with H+) |
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Term
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Definition
glycolysis transamination of Ala deamination of Ser, Cys
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Term
| What makes up pyruvate DH complex |
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Definition
decarboxylase- eliminates CO2 transacetylase- adds CoA onto acetyl dihydrolipoyl dehydrogenase- reduces NAD to NADH
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Term
| Function of fattyl acyl CoA synthetase |
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Definition
converts FA to FA-CoA via ATP hydrolysis to AMP + PP (specific for short, medium, and long chain FA's) PPi will be made into 2 P via pyrophosphatase (makes rxn irreversible)
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Term
Location of fatty acyl CoA synthetases |
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Definition
outer mit. membrane ER membrane peroxisome
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Term
Describe Dietary need for carnitine |
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Definition
| there is no dietary need; we can make all the carnitine we need |
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Term
| Steps in carnitine transferase |
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Definition
at outer mitochondrial membrane, fatty acyl-CoA combine with carnitine to create fatty acyl-carnitine and CoA via ATPase fatty acyl-carnintine move out via translocase antiport with carnitine in mitochondrial matrix, FA is transfered back to CoA to make fatty acyl-CoA via fatty acyl-CoA carnitine transferase II
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Term
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Definition
fatty acyl-CoA converted to enoyl CoA and creates FADH2 via fattyl acyl-CoA dehydrogenase enoyl-CoA and water form beta hydroxylacy-CoA via enoyl-CoA hydratase beta hydroxyacyl-CoA converted to beta ketoacyl-CoA and NADH via beta hydroxyacyl-CoA dehydrogenase beta hydroxyacyl-CoA and CoA-SH combine to form acetyl CoA and fatty acyl-CoA (LOST TWO CARBONS) via beta keto thiolase
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Term
| Overall products of beta oxidation |
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Definition
1 NADH 1 FADH2 1 acetyl CoA 1 fatty acyl-CoA (two carbons shorter) |
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Term
| Eventually, if we keep going through beta oxidation on one fatty acid, when we get to a four carbon chain, how does this change are products |
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Definition
| instead of a fatty acyl Coa, we would get two acetyl-CoA's |
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Term
| Equation to find out number of acetyl CoA's produced by even numbered FA's as well as the number of sequences |
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Definition
#C/2 = #acetyl-CoA #C/2-1 = # sequences |
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Term
| Effect of every double bond present on the end products of beta oxidation |
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Definition
| there is one less flavoprotein (there is already a double bond there, so we can oxidize) |
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Term
| Fate of odd numbered FA's in beta oxidation |
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Definition
| continue breaking down until you form acetyl CoA and propionyl CoA (cant undergo beta oxidation) |
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Term
| Steps of metabolism of propionyl-CoA |
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Definition
via propionyl-CoA carboxylase hydrolysis of ATP (coenzyme: biotin), add CO2 to propionyl-CoA producing ADP/P and methyl malonyl-CoA methyl malonyl-CoA converted to succinyl CoA via methyl malonyl-CoA mutase (coenzyme: Adenosyl-B12) moves carboxyl groop to end of chain
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Term
| Cause of methyl malonic aciduria? |
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Definition
if you are deficient in adenoyl-B12 (coenzyme for methyl malonyl-CoA mutase), causes a build up in methyl-malonyl-CoA leads to ties up CoA if CoA tied up, it decreases if it decrease, we decrease beta oxidation however, body hydrolyzes off CoA it to methyl malonic acid that is excreted in the urine
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Term
| Location of enzymes in propionyl CoA path |
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Definition
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Term
| What type of FA's tend to undergo beta oxidation in the peroxisome membrane? |
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Definition
very long chain FA's (more than 22 C's) |
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Term
Unique features of peroxisome beta oxidation |
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Definition
instead of using a dehydrogenase to produce enoyl CoA and FADH2, we use the oxidase (produces peroxide from oxygen) beta oxidation stops at C8: octanoyl-CoA the end products: octanoyl-CoA and acetyl-CoA
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Term
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Definition
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Term
During what body state are ketone bodies made? |
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Definition
| during starvation (36-48 hrs) |
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Term
| Where are ketone bodies made in the body? what is there fate after that? |
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Definition
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Term
| Clinical application- effect of build up of acetone |
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Definition
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Term
| Function of beta hydroxybutyrate dehydrogenase |
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Definition
| oxidize beta HOB to acetoacetic acid |
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Term
Oxidation rxn of ketone bodies starting from Beta HOB |
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Definition
beta hydroxybutyric acid oxidized to produce acetoacetic acid and NADH via beta hydroxybutyric acid dehydrogenase acetoacetic acid and succinyl-CoA combine to form succinate and acetoacetyl-CoA via transferase acetoacetyl-CoA combine with CoA-SH produce two acetyl-CoA's via thiolase acetyl-CoA go into TCA cycle
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Term
| location of ketone body oxidation in cell |
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Definition
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Term
| Number of ATP's produced from beta-HOB? acetoacetate? |
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Definition
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