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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

25
______condenses fatty acid with coa, with simultaneous hydrolysis of ATP to AMP+PP
ACyl-Coa synthetase
26
what reaction moving the oxidation process forward?
hydrolysis of pp.
27
four key repeated reaction for b-oxdiation
oxdiation, hydration, oxidation, thiolysis
28
net yield of ATP per palmitate ?
106 atp
29
how many ATP expended to activate palmitate?
2
30
acetyl coa from FA oxidation (2Carbon) are lost as ___ in ____cycle
CO2 , TCA cycle | you must maintain blood glucose level
31
reducing power for beta-oxidation?
NADH
32
~50% FA in diet are unsaturated-double bonds must be?
isomerized and reduced
33
odd-chain FA - 3C propionyl Coa. what happen in b-oxidation?
carboxylated to 4C methylmalonyl coa | then --> succinyl coa --> TCA cycle
34
what happens to medium chain FA?
used up immediately
35
carnitine ___only FA from perioxisome
exports ( not involved in import)
36
where detoxification of FA- like compounds- produces dicarboxylic acid can be excreted. what kind of oxidation and where this occurs?
w-oxidation in ER microsome
37
a-oxidation occurs where?
peroxisomes with some branched FA | must remove first carbon
38
what disease is related to a-oxidation?
refsum disease - genetic defect in a-oxidation --> ataxia, neuropathy
39
patient deficient in this transporter have hypoglycemia
carnitine | treat with carnitine supplement
40
what is the first step of b-oxidation?
acyl coa DH
41
deficiency in acyl coa Dehydrogenase
dicarboxylic aciduria (build up from TCA )
42
defects in ___ one of the most common inborn error
MCAD | medium chain acyl coa dehydrogenase
43
where and what is used to make ketone body
acetyl coa in LIVER
44
where KB is converted back to acetyl coa -> TCA
Skeletal muscle/tissues
45
what are the preferred/normal energy source for kidney cortex cells and cardiac muscle?
acetoacetate and beta-hydroxybutyrate
46
where KB synthesis occur?
only in mitochondria matrix
47
can ketone body transport in blood without albumin/other binding protein?
Yes. easily.
48
acetoacetyl coa --> HMG-Coa
HMG-CoA synthase
49
limiting step for KB synthesis
HMG-CoA synthase
50
how KB can be converted to TCA cycle?
aceoacetate --> acetoacetyl coa | by b-ketoacylcoa transferase
51
what are interchangable for KB
Acetoacetate and b-hydroxybutyrate | in normal, 1:1
52
DM patient with KB this leads to?
excess of acetyl Coa excess KB Hyperglycemia -> dehydration -> reduce excretion of KB -> ketoacidosis
53
which tissue/cell use KB as main energy source?
intestinal mucosa cells adopocytes brain under prolonged starvation
54
90mg/100ml =
5mM
55
60mg/100ml=
3.3mM
56
glucose < __mM leads to coma, death
2.2mM

CMBM test 2 (7 decks)

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