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Biochemistry FA > Fatty Acid Metabolism / Ketone Bodies / lipid transport > Flashcards

Fatty Acid Metabolism / Ketone Bodies / lipid transport Flashcards

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1
Q

Long-chain fatty degradation requires

A

Carnitine dependent transport into mitochondrial matric

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1
Q

Inhibitor of carnitine acytransferase

A

Malonyl-coa

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2
Q

Steps of long chain degradation (and location

A
  1. Fatty acid + coa –> Fatty acyl - coa (fatty acid coa synthase) (cytoplasm)
  2. Carnitine dependent transportof Fatty acyl - coa into mitochondria
  3. β-oxidation –> Fatty acyl - coa –> Acetyl coa (Acyl CoA dehydrogenase)
  4. Acetyl coa –> Ketone bodies and TCA cycle
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4
Q

Systemic 1ry Carnitine deficiency - symptoms

A
  1. Weakness
  2. Hypotonia
  3. Hypoketotic hypoglycemia
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4
Q

Acyl-coa dehydrogenase

A

Initial enzyme for β-oxidation

FAD to FADH2

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5
Q

Systemic 1ry Carnitine deficiency - pathophysiology

A

Inherited defect in transport of long chain fatty acids into the mitochondria –> toxic accumulation

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7
Q

Medium-chain acyl-coa dehydrogenase deficiency - pathophysiology / mode of inheritance / presentation

A

AR disorder of fatty acid oxidation –> decreased ability to break down fatty acids into acetyl coa –> accumulation of 8- to 10 - carbon fatty acyl carnites and hypoketotic hypoglycemia
may present in infancy or early childhood with vomiting, lethargy, coma + liver dysfunction –> can leat to sudden death

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8
Q

Mechanism of hypoglycemia in acyl-coa dehydrogenase deficiency

A

Acetyl coa is an +allosteric regulator of pyruvate carboxylase

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8
Q

Ketogenesis fuel and purpose (and location)

A

N the liver, fatty acids and amino acids are metabolyized to acetoacetate and β hydroxybutyrate (to be used in muscle and brain)

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10
Q

Ketogenesis fuel and purpose (and location)

A

In the liver, fatty acids and amino acids are metabolyized to acetoacetate and β hydroxybutyrate (to be used in muscle and brain)

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11
Q

Breath with ketones

A

Smells loke acetone (fruity odor)

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12
Q

types of ketone bodies (and urine test)

A

types: acetoacetate and β hydroxybutyrate

Urine test does not detect β hydroxybutyrate

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12
Q

Ketogenesis in alcoholism

A

Excess NADH shunts oxaloacetate to malate . Buildup of acetyl coa which shunts glucose and FFA toward the production of ketone bodies

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13
Q

3 main situations of ketogenesis (why)

A
  1. Prolonged starvation (depletion of oxaloacetate for gluconeogenesis)
  2. Diabetic ketoacidosis (depletion of oxaloacetate for gluconeogenesis)
  3. Alcoholism (excess NADH shunts oxaloacetate to malate
    - -> buildup of acetyl-coa
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14
Q

Ketogenesis in prolonged starvation and diabetic ketogenesis

A

Oxaloacetate is depleted for gluconeogenesis. Buildup of acetyl coa which shunts glucose and FFA toward the production of ketone bodies

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16
Q

Ketogenesis in the liver - steps and metabolism

A

FA, aminoacids –> Acetyl-Coa –> HMG-CoA (HMG-CoA synthase) –> Autoacetate –> β-Hydroxybutyrate –> acetone (blood) –> expired by lungs
at extra hepatic tissue: β-Hydroxybutyrat –> acetoacetate –> Acetoacetyl-Coa (through TCA) –> Acetyl - Coa –> TCA

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17
Q

1g carbohydrate, 1g fat, 1g protein

A

fat –> 4 kca, carbohydrate –> 4 kca, alcohol –> 9Kcal

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17
Q

Metabolic priorities for fasting and starvation

A

Supply sufficient glucose to the brain and RBC and to preserve protein

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18
Q

energy source at exercise (time)

A

Ovreal performance: 0-1min declining and after plateau (peak at 0)
ATP: 0-2 sec (peak at 0)
Creatine phosphate: 0-10 sec (peak at 2)
aerobic metabolism: O-… (peak at 50 sec and then plateau)
Anaerobic metabolism: 0-1mon (peak at 25 sec)

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20
Q

Fed state (after a meal regulation) mechanism / regulation

A

Glycolysis and aerobic respiration

Insulin stimulates storage of lipids, proteins, glycogen

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21
Q

Fasting state (between meals) mechanism / regulation

A

Major: hepatic glycogenolysis
Minor: hepatic gluconeogenesis, adipose release of FFA
Glucagon, adrenaline stimulate use of fuel reserve

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22
Q

Fasting state (between meals) mechanism is regulated by

A

Glucagon, adrenaline stimulate use of fuel reserve

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22
Q

Glycogen serve depleted after how long

A

1 day

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23
Q

RBC cannot use ketones because

A

They lack mitochondria

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24
Starvation after 3 days
``` Adipose stores (ketone bodies become the main source) After these are depleted, vital protein degration leading to organ faillure and death ```
25
Starvation day 1-3 Blood glucose maintained by
1. hepatic gluconeogenesis 2. Adipose relase of FFA 3. Muscle and liver, which shift fuel use from glucose to FFA 4. Hepatic gluconeogenesis from peripheral tissue lactate and alanine, and from adipose tissue glycerol and propionyl coa (from odd-chain FFA)
26
What does determine survival time at starvation
Amount of excess stores
27
Cholesterol synthesis rate limiting step is catalyzed by
HMG-CoA reductase
28
HMG-CoA reductase reaction
HMG-CoA to mevalonate
29
HMG-CoA regulation
Insulin+ Thyroxine + Cholesterol - Glucagon -
30
LCAT
lecithin cholesterol acyltransferase
31
2/3 of plasma cholesterol is esterified by
Lecithin cholesterol acyltransferase (LCAT)
33
LCAT reaction
Cholesterol to cholesterol ester
34
Statins mechanism of action
HMG-CoA reductase inhibitor
35
Cholesterol use
needed to 1. maintain cell membrane integrity, and to | 2. synthesize bile acid, 3. steroids, and 4. vit D
36
CETP
Cholesterol ester transfer protein
37
CETP function
Mediates transfer of cholesterol esters to other lipoprotein particles and TGs to HDL
38
type of lipases
1. Pancreatic lipase 2. Lipoprotein lipase (LPL) 3. Hepatic TG lipase 4. Hormone sensitive lipase
39
type of lipases and their action
1. Pancreatic lipase --> Degradation of dietary triglycerides in small intestine 2. Lipoprotein lipase (LPL) --> Degradation of TG circulating In chylomicrons and VLDLs. Found on vascular endothelial surface 3. Hepatic TG lipase --> Degradation of TG remaining in IDL 4. Hormone sensitive lipase --> Degradation of TG stored in adipocytes
40
Nascent HDL is produced from
Liver, intestine
41
Nascent to mature HDL - enzyme
Lecithin cholesterol acyltransferase (LCAT)
42
mature HDL - next step
CETP (Cholesterol ester transfer protein): mediates transfer of cholesterol esters to other lipoproteins particles (to VLDL, IDL, LDL)
43
Major apolipopoteins - types
E, A-I, C-II, B-48, B-100
44
Lipd particles
1. Chylomycron 2. Chylomycron remnants 3. VLDL 4. IDL 5. HDL 6. LDL
45
Apolipoprotein E function
Mediates remnant uptake
46
Apolipoprotein E is found at (particles)
1. Chylomycron 2. Chylomycron remnants 3. VLDL 4. IDL 5. HDL (all except LDL)
47
Apolipoprotein A-1 function
Activates Lecithin cholesterol acyltransferase (LCAT) -- Cholesterol to cholesterol ester
48
Apolipoprotein A-1 is founded
Chylomicrons | HDL
49
Apolipoprotein C-II function
Lipoprotein lipase cofactor
50
Apolipoprotein C-II is founded
Chylomicrons, VLDL, HDL
51
Apolipoprotein B-48 function
Mediates chylomicrons secretion
52
Apolipoprotein B-48 is founded
1. Chylomycron | 2. Chylomycron remnants
53
B100 function
Binds LDL receptor | Composition and secretion of VLDL
54
B100 is founded
VLDL IDL LDL
55
Lipoproteins are composed of
Varying proportions of cholesterol, TGs and phospholipids
56
Lipoproteins that carry the most cholesterol
LDL | HDL
57
Transports cholesterol from liver to tissues
LDL
58
Transports cholesterol from tissues to the liver
HDL
59
Chylomicrons function / mechanism / source
secreted by Intestinal epithelial cells --> Deliver dietary TGs to peripheral tissue and becomes chylomicron remnants (by LPL) --> Chylomicron remnants (mostly depleted of their TGs) --> Deliver cholesterol to liver
60
VLDL function / source
secreted by liver --> Delivers hepatic TGs to peripheral tissue
61
IDL function / source
formed in the degradation of VLDL (by LPL) --> Delivers TGs and cholesterol to liver
62
LDL - function / source
Formed in the degradation of IDL (by HL in the liver and the peripheral tissue). Delivers hepatic cholesterol to peripheral tissues --> taken up by target cells via receptor-mediated endocytosis
63
HDL function
1. Cholesterol transport from cholesterol transport from peripheral tissue to liver 2. Act as a repository for apoC and apoE (needed for chylomicrons and VLDL metabolism)
64
......increases HDL synthesis
Alcohol
65
HDL is secreted from
Liver | Intestine
66
Familial dyslipidemias - types and mode of inheritance
- type 1 (Hyperchylomicronemia) - AR - type 2a (hypercholesterolemia) - AD - type 4 (Hypertriglyceridemia) - AD
67
Familial dyslipidemias - type 1 (Hyperchylomicronemia) - pathogenesis
LPL or APO C-II deficiency
68
Familial dyslipidemias - type 1 (Hyperchylomicronemia) - labs
- Increased 1. Chylomycrons, 2. TG, 3. cholesterol in blood | - Creamy layer is supernatant
69
Familial dyslipidemias - type 1 (Hyperchylomicronemia) - clinical presentation
1. Pancreatitis 2. Hepatosplenomegaly 3. eruptive/pruritic xanthomas (NO HIGH RISK FOR ATHEROSCLEROSIS)
70
Familial dyslipidemias - type 2a (hypercholesterolemia) - pathogenesis
Absent or defective LDL receptors
71
Familial dyslipidemias - type 2a (hypercholesterolemia) - lab
High 1. LDL 2. cholesterol
72
Familial dyslipidemias - type 2a (hypercholesterolemia) - values of cholesterol
heterozygotes --> 300mg/dl | homozygous --> 700+ mg/dl
73
Familial dyslipidemias - type 2a (hypercholesterolemia) - heterozygous vs homozygous according to frequency and values of cholesterol
heterozygotes --> 1:500 --> 300mg/dl | homozygous --> very rare --> 700+ mg/dl
74
Familial dyslipidemias - type 2a (hypercholesterolemia) - clinical presentation
- accelerated atherosclerosis (may have MI before 20) - tendon (Achilles) xanthomas - corneal arcus
75
Familial dyslipidemias - type 4 (Hypertriglyceridemia) - pathogenesis
Hepatic overproduction of VLDL
76
Familial dyslipidemias - type 4 (Hypertriglyceridemia) - labs
- High 1. VLDL, 2. TG | - Hypertriglyceridemia (more than 1000)
77
Familial dyslipidemias - type 4 (Hypertriglyceridemia) - clinical presentation
pancreatitis
78
Familial dyslipidemias - types and pathogenesis (and mode of inheritance)
- type 1 (Hyperchylomicronemia) - AR --> LPL or apoC-II deficiency - type 2a (hypercholesterolemia) - AD --> Absent or deficient LDL receptors - type 4 (Hypertriglyceridemia) - AD --> Hepatic overproduction of VLDL
79
Familial dyslipidemias - types and clinical presentation
- type 1 (Hyperchylomicronemia) --> pancreatitis, hepatosplenomegaly, eruptive/pruritic xanthomas - type 2a (hypercholesterolemia) --> accelerated atherosclerosis (may have MI before 20), tendon (Achilles) xanthomas, corneal arcus - type 4 (Hypertriglyceridemia) --> pancreatitis
80
Fatty acid metabolism 1ry occurs in
1. liver 2. lactating mammary glands 3. adipose tissue
81
fatty acid synthesis
citrate (mit) --> to cytoplasm through mit membrane --> acetyl-coa (ATP citrate) acetyl coa + Biotin + CO2 --> Malonyl-CoA --> fatty acid synthesis (palmitate, a 16C FA)
82
Medium-chain acyl-coa dehydrogenase deficiency - treatment
treat by avoiding fasting

Biochemistry FA (16 decks)

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