Term
| what is the structure of a FA? |
|
Definition
| long hydrocarbon chain and a terminal carboxylate |
|
|
Term
| which is a more efficient form of energy: TAG or glycogen |
|
Definition
|
|
Term
| what is the major energy source in cardiac and skeletal muscle? |
|
Definition
|
|
Term
| Why does the brain not utilize FA's? |
|
Definition
| FA's cannot cross the blood brain barrier |
|
|
Term
| what is the site of fatty acid oxidation? |
|
Definition
|
|
Term
| what happens when the hormone lipase is released? |
|
Definition
| in adipose tissue,hydrolysis of TAG to FA's. |
|
|
Term
| when FAs are released into the blood, how does it travel |
|
Definition
|
|
Term
| how are FA's transported inside the cell |
|
Definition
|
|
Term
| in the cells, how is acyl-CoA formed for B-oxidation |
|
Definition
|
|
Term
|
Definition
| protein that coats the lipid droplet |
|
|
Term
| what is the function of phosphorylated perilipin? |
|
Definition
| promotes translocation of lipase to lipid droplet for hydrolysis of FA |
|
|
Term
| what is the effect of glucagon, epinephrine and ACTH on TAG metabolism? |
|
Definition
| it activates the release of free fatty acids from triglycerides |
|
|
Term
| what is the effect of insulin on TAG metabolism |
|
Definition
|
|
Term
| what is the name of the enzyme that catalyzes a FA to FA acyl-CoA |
|
Definition
|
|
Term
| where is FA and FA acyl CoA converted? |
|
Definition
| outer mitochondrial membrane and ER |
|
|
Term
| does the formation of fatty acyl-CoA require ATP? |
|
Definition
| yes. the hydrolysis of two high energy bonds in ATP |
|
|
Term
| what is function of carnitine palmitoyltransferase I? |
|
Definition
| transports FA to the mitochondrial inner membrane |
|
|
Term
| what is the function of translocase? |
|
Definition
| it takes the intermediate Acylcarnitine into the inner membrane. |
|
|
Term
| what effect does malonyl-CoA have CTP and FA oxidation |
|
Definition
| it inhibits CTP, thus preventing FA oxidation in the fed state. |
|
|
Term
| what does the beta oxidation of FA require? |
|
Definition
| successive cleavage of 2 C w/release of acetyl CoA |
|
|
Term
| which FA's diffuse through the membrane |
|
Definition
| short and medium chain FAs |
|
|
Term
| which FA transports through the Carnitine cycle? |
|
Definition
|
|
Term
| which FA transports through the Carnitine cycle? |
|
Definition
|
|
Term
| what are the three substances that are collectively known as ketone bodies? |
|
Definition
| acetoacetone, beta-hydroxybutyrate, acetone |
|
|
Term
| this is an important intermediate in the synthesis of cholesterol |
|
Definition
|
|
Term
| name 4 things that ketone bodies are used for |
|
Definition
| heart, brain, kidney, skeletal muscle |
|
|
Term
| ketone bodies are synthesized to produced |
|
Definition
| 2 acetyl coA going into the TCA cycle |
|
|
Term
| the hydrolysis of triglyceride in adipose tissue produces this, which eventually leads to glucose |
|
Definition
|
|
Term
| what does the liver synthesize glycerol to? by what enzyme? |
|
Definition
| glycerol 3 phosphate by glycerol kinase |
|
|
Term
| what is glycerol 3 phosphate converted to in the liver to undergo gluconeogenesis |
|
Definition
|
|
Term
| where does omega oxidation of FA occur? |
|
Definition
| in the ER with involvement of cytochrome P450 |
|
|
Term
| what is the prevalent pathway after beta oxidation of FA is defective? |
|
Definition
|
|
Term
| in omega oxidation, what is introduced on the first step? |
|
Definition
|
|
Term
| What is the hydroxyl group oxidized to on the omega carbon? |
|
Definition
| aldehyde and then carboxylic acid |
|
|
Term
| what is the end product of omega oxidation? |
|
Definition
|
|
Term
| what is associated with secondary carnitine deficiency? |
|
Definition
| defects in the beta oxidation pathway w/accumulation of acylcarnitines, which are secreted in teh urine |
|
|
Term
| what are some clinical manifestations of primary carnitine deficiencies? |
|
Definition
| muscle cramping, muscle weakness |
|
|
Term
| what is caused by a primary carnitine deficiency |
|
Definition
| defect of carnitine transporter |
|
|
Term
|
Definition
| mutation of the CPTII gene |
|
|
Term
| what are the clinical manifestations of CPT deficiency? |
|
Definition
| muscle weakness during prolonged exercise with loss of myoglobin in the urine |
|
|
Term
| how are translocase deficiencies treated? |
|
Definition
| avoidance of fasting and diet supplemented by medium chain FA |
|
|
Term
| what are the clinical symptoms of translocase deficiency |
|
Definition
| hypoglycemic coma, hyperammoniemia, muscle weakness, cardiomyopathy |
|
|
Term
| these inherited disorders affect the first reaction in the B-oxidation of FA |
|
Definition
| acyl CoA dehydrogenase deficiency |
|
|
Term
| what type of dehydrogenases are affected in acyl-CoA dehydrogenases? |
|
Definition
| short, medium, and very long chain dehydrogenases |
|
|
Term
| what is hypoketotic hypoglycemia due to? |
|
Definition
| a block of B oxidation of FA's which slows gluconeogenesis in the liver. |
|
|
Term
| what is dicarboxylic aciduria due to? |
|
Definition
| accumulation of FA that enter alternative degration pathways such as omega oxidiation. excessive secretion of dicarboxylic acids |
|
|
Term
| what is sudden infant death due to? |
|
Definition
|
|
Term
| where does oxidation of very long chain FA occur? |
|
Definition
|
|
Term
|
Definition
| failure to oxidize long chain FA |
|
|
Term
| What is Zellweger syndrome |
|
Definition
| inability to make peroxisomes |
|
|
Term
| Patients w/zellweger syndrome and XALD have accumulation of what in the blood? |
|
Definition
|
|
Term
| This antidiabetic drug causes a marked increase in |
|
Definition
|
|
Term
| beta oxidation in the peroxisome of very long chain FA produces |
|
Definition
|
|
Term
| With odd-chain FA metabolism via Beta oxidation, what is the product most likely to be? |
|
Definition
|
|
Term
| where does Alpha oxidation of FA occur? |
|
Definition
ER, mitochondria, peroxisomes.
also involves mono oxigenases (P450 family) that use NADH or NADPH |
|
|
Term
| what is alhpa oxidation important for? |
|
Definition
| catabolism of branched chain FA |
|
|
Term
| what are the products of alpha oxidation of FA? |
|
Definition
| 3 mols of propionyl-CoA, acetyl CoA and one molecule of isobutyrl CoA |
|
|
Term
| what is involved in the reaction of phytanoyl CoA being hydroxylated? |
|
Definition
| a-ketoglutarate, ascorbate, and iron |
|
|
Term
| Refsum's disease is characterized by? |
|
Definition
| lack of a-hydroxylating enzyme and accumulate phytanic acid |
|
|
Term
| what will insulin stimulate in the fed state? |
|
Definition
| FA synthase, NADPH malic dehydrogenase, and acetyl CoA carboxylase |
|
|
Term
| what enzymes do insulin stimulate in the fed state |
|
Definition
glut 4 translocation, glucose breakdown to glycerol 3-phosphate for synthesis of TAG.
turns on phosphoprotein phosphatase (removes phosphate from acetyl CoA and activates enzyme)
also stimulates pentose pathway w/generation of NADPH |
|
|
Term
| what is the effect of glucagon in the fasted state? |
|
Definition
| they inhibit acetyl-coA carboxylase, decreasing FA synthesis |
|
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