Fatty Acid Metabolism Flashcards

(49 cards)

1
Q

What are three sources of lipid?

A

Fats consumed in the diet

Fats stored in cells as lipid droplets

Fats synthesized in one organ (liver) for transport to another organ

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2
Q

What is the function of acid resistant lingual lipases?

A

Secreted from glands under the tongue

Begins fat digestion in the stomach

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3
Q

What are gastric lipases?

A

Lipases that act in the stomach to digest fats

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4
Q

What is the function of pancreatic lipases?

A

To removie FA at carbons 1 and 3 of triglyceride

*different lipases for each

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5
Q

What is the function of colipase?

A

Anchors pancreatic lipase at the lipid-aqueous interface

Secreted as zymogen

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6
Q

What is the function of Cholesterol esterase?

A

Revmoves FA from cholesterol

Secreted from pancreas

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7
Q

How are phospholipids digested?

A

Phospholipase A2 removes FA from one carbon

Lysophospholipase removes FA at C1

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8
Q

What is the product of phospholipid digestion?

A

Glycerylphosphorylcholine

Exreted, further degraded, or absorbed

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9
Q

What is the function of Cholecystokinin (CCK)?

A

Hormone released from jejunum and duodenum

Acts on Gall bladder to release bile

Acts on pancreas to release enzymes from exocrine cells

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10
Q

What is the function of Secretin?

A

Produced in response to low pH of chyme

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11
Q

What are two consequences of lipid malabsorption?

A

Steatorrhea

Deficiency of lipid soluble vitamins ADEK

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12
Q

What is the function of Apolipoprotein C-II?

A

Activates lipoprotein lipase located on blood vessels

Part of chylomicrons

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13
Q

How are chylomicrons used by tissues?

A

Broken down in capillaries of skeletal muscle or adipose

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14
Q

How are Free fatty acids used by tissues?

A

Fuel or storage or transported to other cells

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15
Q

What is glycerol used for?

A

Used by liver to produce glycerol-3-phosphate for glycolysis or gluconeogenesis

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16
Q

How are chylomicron remnants used by the cell?

A

Endocytosed by liver and hydrolized.

Cholesterol and nitrogenous bases can be recycled

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17
Q

How are triglycerides transported from the lumen to the blood?

A

Broken down in lumen

Re-synthesized within the intestinal cell

TGs are excreted and transported in chylomicrons

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18
Q

What complications of CF are related to fat metabolism?

A

Growth Failure

Bone Disease

Deficiency of fat soluble vitamins

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19
Q

What is medium-chain acyl-CoA dehydrogenase (MCAD) deficiency?

A

Most common genetic defect in FA metabolism in US and Northern Europe

Mutation in enzyme

Causes vomiting, lethargy, hypoketosis, hypoglycemia

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20
Q

What are the three stages of processing for the utilization of fatty acids?

A

Mobilization

Activation and Transport to the mitochondria

Fatty acids broken down into acetyl-CoA

21
Q

How are storage fatty acids signaled to be mobilized?

A

Hormone signalling triggers cAMP/PKA pathway that activates a triacylglycerol lipasae

22
Q

What is the function of Perilipin?

A

Phosphorylated by PKA, increases access to lipids by permeabilizing the lipid droplet

23
Q

What is the function of Fatty acyl-CoA synthetase?

A

Activates Fatty Acids by attaching CoA via ATP hydrolysis

24
Q

What is the function of Carnitine acyltransferase I?

A

Attaches an activated FA to carnitine for transport into the mitochondria

CoA provides the energy for this reaction

25
What type of fatty acids can cross the mitochondrial membrane without carnitine?
Short or medium chain fatty acids <12 C
26
How is carnitine obtained?
Meat Synthesized from lysine and methionine by liver or kidney
27
What is the rate limiting step for FA oxidation?
Carnitine transport
28
What inhibits CPT-1?
Malonyl CoA
29
What things could result in carnitine deficiency?
Liver disease Malnutrition or vegetarian diet Increased requirement - burns, pregnancy Hemodialysis Congenital deficiency
30
What results from an inability to use LCFA as fuel?
Imparied ability to synthesize glucose furing a fast, possible hypoglycemia, coma and death Impaired ability to sustain exercise
31
What are the three stages of Fatty acid oxidation?
Successive removal of two C units Acetyl groups are oxidized to CO2 in the citric acid cycle Electrons are donated to the mitochondrial respiratory chain to phosphorylate ADP to ATP
32
What is the order of reactions in fatty acid oxidation?
Oxidation-->Hydration-->Oxidation-->Thiolysis
33
What is produced during each round of FA oxidation removing two carbons?
1 FADH2 - first oxidation 1 NADH - second oxidation 1 Acetyl CoA - thiolysis reaction
34
How many ATP are formed from complete oxidation of palmitate (C16)?
106 ATP
35
What type of fatty acid does B-oxidation completely degrade?
Saturated fatty acids with an even number of carbons
36
What are the final products of B-oxidation of odd-number chain fatty acids?
Propionyl CoA and Acetyl CoA
37
What is the fate of Proprionyl CoA after B-oxidation?
Converted to succinyl CoA to enter the Citric acid cycle
38
What is the function of propionyl CoA carboxylase and what does it require?
Forms Methylmalonyl CoA from Propionyl CoA Requires biotin
39
What does L-methylmalonyl--> succinyl CoA isomerization require?
Methylmalonyl CoA Mutase Derivative of Vit B12 as coenzyme
40
What do unsaturated fatty acids with an odd number of double bonds require?
An additional isomerase that converts the cis double bond to a trans double bond
41
What do unsaturated fatty acids with an even number of double bonds require?
Reductase and isomerase
42
What is Refsum Disease?
Genetic deficiency in the peroxisomal enzyme responsible for one of the initial steps in the oxidation of phytanic acid
43
What are the symptoms of Refsum disease?
Retinities Pigmentosa Progressive peripheral neuropathy Skeletal malformation Sever motor weakness
44
Where are very long, or branched chain fatty acids oxidized?
Peroxisomes
45
What are the four steps in a-oxidation for branched chains?
Activation with CoA CoA and Formic acid released Dehydrogenation Activation with CoA, then B-oxidation releases propional CoA
46
What is Ketosis?
Ketone bodies are formed from Acetyl CoA when fat degredation predominates
47
What ketone bodies are formed during ketosis?
Acetoacetate 3-hydroxybutyrate Acetone
48
How is ketogenesis regulated?
Entry of FA into oxidative pathway controlled by CPT-1 As level of FFA increases, more FFA converted to ketones
49
How does diabetes cause ketoacidosis?
Absence of insulin means liver cannot provide oxaloacetate Insulin normally decreases fatty acid metabolism Result is liver produces large amount of ketone bodies which are moderately strong acids