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MSS - MSK Exam 3 > Energy Metabolism in Muscle > Flashcards

Flashcards in Energy Metabolism in Muscle Deck (46)
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31

omega oxidation

of fatty acids

during prolonged fasting
-in liver peroxisome

forms DCAs which go through mitochondrial beta-oxidation

32

difference between beta ox and peroxisome ox

beta two separate enzymes
peroxisome ox one multifunction enzyme protein

33

zellweger syndrome

accumulation of long chain fatty acids
peroxisomal disorder

34

aderenoleukodystrophy

accumulation of long chain fatty acids
peroxisomal disorder

35

indicatin of omega ox?

DCAs in urine

36

inborn errors of fatty acid oxidation?

DCAs and acylglycine in urine
acylcarnitine derivatives in serum

37

cori cycle

recycles lactic acid

more efficient when muscle activity stops
oxygen debt can be made up

lactate > liver
-converted to pyruvate then to glucose (requires ATP)

38

what favors lactate production in skeletal muscle?

high NADH/NAD + ratio

intense exercise, increased lactate

drop in pH
-cramps

39

lactic acidosis sign of what?

MI
pulmonary embolism
uncontrolled embolism
uncontrolled hemorrhage

**any time we can't get blood to tissue

40

regeneration of NAD+

done by lactate formation (anaerobic glycolysis)

41

primary carnitine deficiency syndrome

lack of carnitine within cell (carnitine transporter mutation)
-lipid myopathy


fatty acid oxidation significantly reduced**

42

secondary carnitine deficiency syndrome

carnitine sequestered in form of acyl-carnitine
-carnitine cannot be removed from acyl group

defect in ACT II

elevated levels of acyl carnitine

fatty acid oxidation significantly reduced**

43

fatty acid transport defects

CPT I
CPT II
carnitine:acylcarnitine translocase

44

mitochondrial defects

defects of beta-oxidation enzymes

myopathic symptomes that are usually progressive

45

oxidative phosphorylation system

main source of energy in muscles and other cells

46

fasting?

omega oxidation (DCA production)