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biochemistry I > Fatty Acid Transport and Beta Oxidation - Ch. 30 > Flashcards

Fatty Acid Transport and Beta Oxidation - Ch. 30 Flashcards

1
Q

L carnitine is converted to ____ which contributes to the formation of __.

A

TMAO - transmethyl amine oxide; atherosclerosis -> cardiovascular disease

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2
Q

What do Long chain FA need to enter into the plasma membrane?

A

They need to bind to albumin (proteins that act as a carrier)

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3
Q

Where do long chain fatty acids come from? What is the end goal

A

Adipocytes; convert to acetyl coA and make NADH and FADH2

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4
Q

Long Chain FA are soluble in water. True/False?

A

False, they are insoluble (that is why they need FA binding proteins)

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5
Q

How do FA get activated to be able to enter the outer mitochondrial membrane? What do they turn into that enters the outer membrane (intermembrane space)?

A

Coenzyme A; Fatty Acyl CoA

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6
Q

How does FA get into the inner mitochondrial membrane (inside the middle of the mitochondria)?

A

CoA comes off and carnitine (shuttle into inner mitochondria) replaces it. Then carnitine comes off once inside (back to a fatty acyl CoA)

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7
Q

Serum albumin can bind how many FA? What else can it bind? Why?

A

6; hormones and drugs; bc its nonspecific

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8
Q

FA entry into cells can be ___.

A

regulated (but we wont worry abt this)

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9
Q

How do all FA get into cells? And where could they go into?

A

They use a saturable binding protein and free diffusion (to get into mitochondria, peroxisomes, ER)

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10
Q

Are synthases = synthetases?

A

Synthetases CONSUME ATP, but they are a type of synthase (usually interchangeable)

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11
Q

A Very Long chain is how big (#)?

A

about 14+ but varies

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12
Q

How many isoforms of Fatty acyl CoA synthetase exist?

A

4

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13
Q

Acetyl CoA gets used where/how?

A

To make KB in the liver, and make more NADH, FADH2, 2CO2 in the TCA cycle

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14
Q

The intermediate between FA and fatty acyl CoA is? How is this possible?

A

Fatty acyl AMP (covalent) + pyrophosphate ; FA synthetase uses a nucleophile oxygen from carboxyl group to attack the FA oxygen

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15
Q

Activating FA CoA requires how much ATP?

A

2ATP (because AMP is generated)

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16
Q

After a Fatty acyl CoA has been activated and crosses the plasma membrane of the cell, where do MC Fatty acids end up?

A

Medium chain FA are in the matrix

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17
Q

After a Fatty acyl CoA has been activated and crosses the plasma membrane of the cell, where do LC Fatty acids end up?

A

Long chain FA are in the outer layer

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18
Q

After a Fatty acyl CoA has been activated and crosses the plasma membrane of the cell, where do Very long chain FA synthetases interact with them?

A

very long chain- peroxisomes

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19
Q

After a Fatty acyl CoA has been activated and crosses the plasma membrane of the cell, where are Short chain FA synthetases they interact with?

A

In the cytosol

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20
Q

What organelles of the cell are used to make energy from Fatty acids?

A

peroxisome (VLC) and mitochondria (LC, MC)

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21
Q

How do medium chain FA travel into mitochondria?

A

Using monocarboxylate transporters (context of lactate and KB?). they are then activated to fatty acyl coas and do b ox.

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22
Q

What does the ER have to do with FA? (location, function)

A

Not only are LC FA synthases on outer mitochondrial layer, but they are on the ER too! The ER’s job is to store and create membrane lipids

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23
Q

The ER’s job is to store and create membrane lipids . Which ones?

A

phospho and sphingo lipids are membrane lipids, and triacylglycerols are for storage

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24
Q

What is carnitine synthesized from in our bodies? Where else do we get it?

A

From lysine; diet (mostly red meat)

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25
Q

Carnitine deficiencies are rare, T/F?

A

TRUE

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26
Q

Gut microbes generate tri methyl amine from
_-carnitine and ___

A

from L carnitine and choline ( a head group)

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27
Q

An oxidase in the liver converts TMA to TMAO, causing reverse __ transport->___.

A

cholesterol; hypercholesterolemia because of decreased bile acid making

28
Q

How can we prevent atherosclerosis?

A

allicin in garlic, antibiotics

29
Q

The beta oxidation spiral happens in the _. How many acetyl Coa’s are formed (repetition)?

A

mitochondrial matrix; 6 repetitions make 7 total

30
Q

How do you know if a FA got activated to become a acetyl coa

A

2 carbons got cut off (16->14)

31
Q

In the FA oxidization spiral, which carbon is targeted? What enzyme does this?

A

The beta carbon (2nd from the carbonyl C-C-=O-C); Acyl CoA dehydrogenase

32
Q

How many isoforms of acyl CoA exist

A

4 (depends on chain length, vlc, lc, mc, sc)

33
Q

What is the order of the b oxidation spiral steps?

A

oxidation, hydration, oxidation,

34
Q

The first step of the b oxidation cycle yields __. where does it go?

A

FADH2 and 1.5 ATP; FADH2 directly goes into TCA without leaving acyl coa dehydrogenase

35
Q

The third step of b oxidation yields

A

NADH and 2.5 ATP

36
Q

An alkene has how many bonds? An alkyne?

A

2; 3

37
Q

For long chain fatty acids, once they go into the porous outer mitochondrial membrane, they become a substrate for ___. What does this enzyme do?

A

Carnitine: palmitoyl transferase I (CPT I) transferase; it takes off CoA and puts a carnitine in its place

38
Q

What is the antiport process in long chain fatty acid transport?

A

The antiport process is the movement of fatty acyl carnitine in and carnitine:acylcarnitine translocase send it out of the matrix.

39
Q

What is the job of carnitine:palmitoyl transferase II (CPT II)?

A

It takes carnitine off the fatty acyl carnitine and replaces it with CoA. then fatty acyl coa is born! (and can go into beta oxidation)

40
Q

primary CPT I deficiency causes __ in the blood

A

increased blood FA

41
Q

primary CPT II deficiency causes ___

A

elevated blood acylcarnitine, increased blood FA

42
Q

As a FA chain goes through the b oxidation spiral, what happens?

A

they become substrates for different acyl coA dehydrogenases

43
Q

When would thiolysis go in the reverse direction? Why?

A

when lots of acetyl coa (can make acetoacetate in the ketone synthesis pathway or get used in cholesterol synthesis)

44
Q

Unsaturated FA can be beta oxidized using

A

additional enzymes - enoyl CoA isomerase and 2,4 dienoyl coa reductase (idt we need to know)

45
Q

most fatty acids from our diet and in adipose tissue are __. they use __ system.

A

long chain; carnitine transport

46
Q

When we consume fiber, gut bacteria convert it to

A

short chain fatty acids

47
Q

short chain fatty acids examples are

A

acetate, propionate, butyrate

48
Q

short chain fatty acids have benefits such as

A

better immunity and metabolism, food for colonocytes that are ox by liver for energy

49
Q

Which products are rich in medium chain FA?

A

dairy, coconut/palm oil

50
Q

Describe the last repitition of an even chain fatty acid going down the b oxidation spiral

A

4 C chain = butyryl CoA does 1 last repetition of the spiral. the second to last step makes acetoacetyl CoA, and the last step of the spiral is thiolysis, creating 2 CoA molecules

51
Q

How many carbons are left on before the last step of a odd chain beta oxidation spiral? what are we left with after?

A

5; propionyl coa

52
Q

Propionyl coa is a __ process in the __

A

anaplerotic (refilling); tca cycle

53
Q

Proionyl coa gets converted to __ inthe ___ pathway

A

succinyl coa in the vomit pathway

54
Q

Where are FA oxidation for LC happening organ wise?

A

is it the liver? liver adipocytes?

55
Q

FA made outside the liver are used for? what types are these?

A

membranes; VLC, MC, SC

56
Q

MC and SC Fatty acids are not stored in adipose. rather, where do they go?

A

they get moved directly to liver or other organs for oxidation

57
Q

medium chain fatty acyl CoA synthetase also recognizes

A

drugs with carboxyl groups

58
Q

You can do some __ with odd chain fatty acids because

A

gluconeogenesis; succinyl coa is made and fed into the tca cycle

59
Q

The first step of propionyl coa -> succinyl coa requires what enzyme and substrate?

A

propionyl coa carboxylase; biotin

60
Q

The second step of propionyl coa -> succinyl coa requires what enzyme and substrate?

A

methylmalonyl coa epimerase (d methylmalonyl coa to l methylmalonyl coa)

61
Q

The third step of propionyl coa -> succinyl coa requires what enzyme and substrate?

A

methylmalonyl coa mutase needs b12; l methylmalonyl coa->succinyl coa

62
Q

biotin is a substrate that activates __ for a carboxylase enzyme to do its job

A

co2 (inside hco3-?)

63
Q

Why is methylmalonyl coa mutase significant?

A

1 of 2 enzymes that need b12

64
Q

How are acylglycines targeted?

A

they are a marker for elimination (esp in liver->GI->feces or resorb by renal sys->urine)

65
Q

A defect in fatty acyl coa dehydrogenase causes … to accumulate; where ?

A

accumulated fatty acyl coa’s and fatty acyl carnitines to show up in the urine and serum

66
Q

The CoA adduct made by MC acyl CoA synthetase is turned into acylglycine by which enzyme

A

glycine N acyltransferase

67
Q

Sometimes FAcyl CoA that accumulate with CPT disorders can be substrates for __. Which direction?

A

CPT II in the direction of making carnitines instead of break down (reverse rxn). This reverse reaction generates CoA-SH to use elsewhere for TCA

biochemistry I (11 decks)

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