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Basic Biochemistry > 35. beta oxidation > Flashcards

35. beta oxidation Flashcards

1
Q

removal of acetyl CoA from the ends of fatty acids

A

beta-oxidation

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2
Q

products of beta-oxidation

A
  • 8 Acetyl CoA -> citric acid cycle
  • 7 NADH -> ETC
  • 7 FADH2 -> ETC
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3
Q

where does beta oxidation occur

A

muscle
liver
•mitochondria

•fatty acid activation occurs in the cytosol

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4
Q

substrate of beta-oxidation

A

palmitate

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5
Q

rate limiting step in beta oxidation

A

translocation of fatty acyl coa from cytosol to the mitochondria
Enz : carnitine-palmitoyl transferase

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6
Q

Transport of fatty acyl coa to the mitochondria involves

A
  1. activation of the fatty acid ≈ fatty acyl synthetase
  2. attachment of fatty acyl to carnitine in the outer mitochondrial membrane ≈ carnitine acyltransferase-1
  3. fatty acyl-carnitine is shuttled through the inner membrane
  4. transfer of fatty acyl group back to a CoA in mitochondrial matrix
    ≈ carnitine acyltransferase-2
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7
Q

this enzyme activates the fatty acid

A

fatty acyl synthetase

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8
Q

degradation of faty acyl coa are a sequence of these steps, repeated 7 times

A

Oxidation —> Hydration —> Oxidation —> Thiolysis

OHOT

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9
Q

enzymes collectively known as fatty acid oxidase

A 
(4)
•fatty acyl coa dehydrogenase
•∆2 enol coa hydratase
•3-hydroxyacyl coa dehydrogenase
•thiolase
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10
Q

how much ATP in NADH

A

2.5 ATP each

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11
Q

how much ATP in FADH2

A

1.5 ATP each

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12
Q

how much ATP in acetyl coa

A

10 ATP each

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13
Q

Net atp yield of palmitate

A

108 ATP -2 ATP (activation)

= 106 ATP

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14
Q

oxidize very long chains of fatty acids (C20, C22)

A

peroxisomes

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15
Q

oxidation of unsaturated FAs require this additional enzyme

A

3,2 enoyl-CoA isomerase

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16
Q

oxidation of FA with an odd number of carbon atoms will yield

A

acetyl Coa and a molecule of propionyl CoA

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17
Q

propionyl CoA is converted to ____, a TCA intermediate

A

succinyl CoA

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18
Q

propionyl CoA carboxylase requires

A

biotin

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19
Q

methylmalonyl CoA mutase requires

A

vit B12

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20
Q

rate limiting enzyme of lipogenesis

A

acetyl CoA carboxylase

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21
Q

subcellular location of lipogenesis

A

cytosol

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22
Q

subcellular location of beta oxidation

A

mitochondria

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23
Q

transport mechanism of lipogenesis

A

citrate shuttle

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24
Q

transport mechanism of beta-oxidation

A

carnitine shuttle

25
Q

repetitive steps of lipogenesis

A
  • condensation
  • reduction
  • dehydration
  • reduction

CRDR

26
Q

product of lipogenesis

27
Q

product of beta oxidation

A

8 acetyl CoA
7 NADH
7FADH2

28
Q

activator of lipogenesis

29
Q

inhibitor of lipogenesis

A

long-chain fatty acetyl CoA

30
Q

inhibitor of beta oxidation

A

malonyl coa

31
Q

hormonal state favoring lipogenesis

A

high insulin

32
Q

hormonal state favoring beta -oxidation

A

high glucagon

33
Q

lipid malabsorption manifests as

A

steatorrhea

34
Q

lipid malabsorption results to

A

fat soluble vitamin and essential fatty acids deficiency

35
Q

possible causes of lipid malabsorption

A 
liver disease
pancreatic disease
cholelithiasis 
shortened bowel
intestinal mucosal defects
36
Q

deficiency of essential FA results in

A 
ichthyosis (scaly dermatitis)
hair loss 
poor wound healing
visual
neurologic abnormalities
37
Q

carnitine deficiency causes

A 
preterm infants
liver disease
malnutrition or strictly vegetarian diet
pregnancy 
severe infections 
burns 
trauma
hemodialysis
38
Q

presents with
•hypoglycemia due to impaired fatty acid oxidation
•lipid accumulation with muscular weakness

A

carnitine deficiency

39
Q

tx of carnitine deficiency

A

oral supplementation with carnitine

40
Q

carnitine-palmitoyl transferase I deficiency affects the

41
Q

carnitine-palmitoyl transferase II deficiency affects the

A

cardiac and skeletal muscle

-cardiomyopathy, muscle weakness with myoglobinemia after prolonged exercise

42
Q

leads to dec oxidation of FAs with 6-10 carbons

A

MCAD deficiency

medium chain fatty acyl coa dehydrogenase deficiency

43
Q

most common inborn eror ofFA oxidation

A

MCAD deficiency

medium chain fatty acyl coa dehydrogenase deficiency

44
Q

inheritance pattern of MCAD deficiency

A

autosomal recessive

45
Q

MCAD deficiency

higher indcidence in

A

norther europeans

46
Q

can manifest as
severe hypoglycemia
accumulation of FA in urine
sudden infant death syndrome

A

MCAD deficiency

medium chain fatty acyl coa dehydrogenase deficiency

47
Q

tx of MCAD deficiency

A
  1. avoidance of fastig

2. IV glucose during acute episodes

48
Q

caused by eating unripe fruit of the akee tree

A

jamaican vomiting sickness

49
Q

toxin causing jamaican vomiting sickness

A

hypoglycin

50
Q

hypoglycin inactives

A

medium and short chain acyl CoA dehydrogenase - inhibit beta oxidation

51
Q

this disease is caused by deficiency of phytanoyl hydroxylase that leads to accumulation of phytanic acid

A

Refsum disease

52
Q

Tx of refsum disease

A

diet low in phytanic acid

avoid dairy products, ruminant fat and meat

53
Q
  • presents with neurodegeneration (initial apathy and behavioral change, visual loss, spasticity, ataxia)
  • adrenocortical insufficiecy
  • hypogonadism
A

Adrenoleukodystrophy (ALD)

54
Q

presents with
•liver dysfunction w/ jaundice
•marked mental retardation, weakness, hypotonia
•cranofacial dysmorphism - high forehead, shallow orbits, hypertelorism, high arched palate, abnormal helices of ears, retrognathia
•early death

A

zellweger syndrome

55
Q
  • peripheral neuropathy and ataxia
  • retinitis pigmentosa
  • abnormalities in skin and bones
A

refsum disease

56
Q
  • cerebrohepatorenal syndrome
  • inherited absences of peroxisomes in all tissues
  • marked accumulation of very long chain, saturated, unbranched, fatty acids in the liver, and central nervous system
A

Zellwegger syndrome

57
Q

inability to transport VLCFAs across the peroxisomal membrane leads to accumulation in the brain, adrenals and testes

A

adrenoleukodystrophy

58
Q

tx of ALD

Adrenoleukodystrophy

A

Adrenal hormone replacement therapy

Hematopoetic stem cell transplantation

Basic Biochemistry (50 decks)

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