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CMBM test 2 > jenny beta oxidation of FA > Flashcards

jenny beta oxidation of FA Flashcards

1
Q

no C-C double bonds

A

saturated FA

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2
Q

at least one CC double bond

A

unsaturated FA

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3
Q

one CC double bond

A

monosaturated

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4
Q

two or more CC double

A

polyunsaturated

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5
Q

2-3 carbons

A

short chain

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6
Q

4-12 carbons

A

medium chain

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7
Q

12-20 carbons

A

long chain

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8
Q

more than 20

A

very long chain

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9
Q

fatty acids are stored as neutral lipids called____

A

triaclyglycerols (TGs)

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10
Q

what lipases chop off the fatty acids?

A

triacylglycerol lipases

diacylglycerol and monoacylglyerol lipases

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11
Q

are free fatty acids in cytoplasm?

A

rare, they are bound to glycerol, coenzymeA, or carnitine

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12
Q

in blood where FA is found?

A

Lipoproteins

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13
Q

Blood that circulate, FA is bound to what?

A

serum albumin

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14
Q

this is major serum protein

A

albumin

60% of total plasma protein

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15
Q

where beta-oxidation occur?

A

mitochondrial matrix (longer chain)

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16
Q

where unsaturated FA begin beta-oxidation?

A

in peroxisome

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17
Q

long chain FA converted to what for transport across inner membrane

A

acylcarnitine

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18
Q

how long chain FA is transported thru membrane?

A

carnitine:acylcarnitine translocase (CAT)

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19
Q

What is key regulatory step in beta oxidation?

A

FA –> acylcarnitine

transport across inner membrane by CAT

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20
Q

carrier for FA breakdown

A

acylgroup-s-coa

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21
Q

carrier for FA biosynthesis

A

acyl group- S- ACP

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22
Q

cofactor FA breakdown

A

NAD+/NADH

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23
Q

cofactor- FA synthesis

A

NADP+/NADPH

24
Q

B-oxdiation degrades__ at a time

A

2 Carbon

25
Q

______condenses fatty acid with coa, with simultaneous hydrolysis of ATP to AMP+PP

A

ACyl-Coa synthetase

26
Q

what reaction moving the oxidation process forward?

A

hydrolysis of pp.

27
Q

four key repeated reaction for b-oxdiation

A

oxdiation, hydration, oxidation, thiolysis

28
Q

net yield of ATP per palmitate ?

A

106 atp

29
Q

how many ATP expended to activate palmitate?

A

2

30
Q

acetyl coa from FA oxidation (2Carbon) are lost as ___ in ____cycle

A

CO2 , TCA cycle

you must maintain blood glucose level

31
Q

reducing power for beta-oxidation?

A

NADH

32
Q

~50% FA in diet are unsaturated-double bonds must be?

A

isomerized and reduced

33
Q

odd-chain FA - 3C propionyl Coa. what happen in b-oxidation?

A

carboxylated to 4C methylmalonyl coa

then –> succinyl coa –> TCA cycle

34
Q

what happens to medium chain FA?

A

used up immediately

35
Q

carnitine ___only FA from perioxisome

A

exports ( not involved in import)

36
Q

where detoxification of FA- like compounds- produces dicarboxylic acid can be excreted. what kind of oxidation and where this occurs?

A

w-oxidation in ER microsome

37
Q

a-oxidation occurs where?

A

peroxisomes with some branched FA

must remove first carbon

38
Q

what disease is related to a-oxidation?

A

refsum disease - genetic defect in a-oxidation –> ataxia, neuropathy

39
Q

patient deficient in this transporter have hypoglycemia

A

carnitine

treat with carnitine supplement

40
Q

what is the first step of b-oxidation?

A

acyl coa DH

41
Q

deficiency in acyl coa Dehydrogenase

A

dicarboxylic aciduria (build up from TCA )

42
Q

defects in ___ one of the most common inborn error

A

MCAD

medium chain acyl coa dehydrogenase

43
Q

where and what is used to make ketone body

A

acetyl coa in LIVER

44
Q

where KB is converted back to acetyl coa -> TCA

A

Skeletal muscle/tissues

45
Q

what are the preferred/normal energy source for kidney cortex cells and cardiac muscle?

A

acetoacetate and beta-hydroxybutyrate

46
Q

where KB synthesis occur?

A

only in mitochondria matrix

47
Q

can ketone body transport in blood without albumin/other binding protein?

A

Yes. easily.

48
Q

acetoacetyl coa –> HMG-Coa

A

HMG-CoA synthase

49
Q

limiting step for KB synthesis

A

HMG-CoA synthase

50
Q

how KB can be converted to TCA cycle?

A

aceoacetate –> acetoacetyl coa

by b-ketoacylcoa transferase

51
Q

what are interchangable for KB

A

Acetoacetate and b-hydroxybutyrate

in normal, 1:1

52
Q

DM patient with KB this leads to?

A

excess of acetyl Coa
excess KB
Hyperglycemia -> dehydration -> reduce excretion of KB -> ketoacidosis

53
Q

which tissue/cell use KB as main energy source?

A

intestinal mucosa cells
adopocytes
brain under prolonged starvation

54
Q

90mg/100ml =

A

5mM

55
Q

60mg/100ml=

A

3.3mM

56
Q

glucose < __mM leads to coma, death

A

2.2mM

CMBM test 2 (7 decks)

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