Fatty Acid and TAG Synthesis

Question 1

linoleic acid

linolenic acid

arachidonate

Answer 1

omega 6-pro inflammatroy-SMASH

omega 3-anti-inflammatroy

synthesized from linoloic-omega 6-prostaglanding precursor

Question 2

why cant mammals syntehsize omega 6 or 3

Answer 2

dont have enzyme to introduce double bonds beyond C9 in FA

Question 3

Essential FA def

Answer 3

dry/flaky/scaly/lackluster skin, small bumps, mixed oily and dry skin

Question 4

where are FA synthesized

Answer 4

liver, lactating tissue

-and lesser exxtent adipose

Question 5

what occurs in mito

Answer 5

FA ox, acetyl Coa production, ketone body syn, FA elonation

Question 6

wha toccurs in ER

Answer 6

phospholipid synthesis
late stage sterol syn
FA elongation
FA desaturation

Question 7

what occurs in cytosol

Answer 7

NADPH production-HMP/malic enzyme
NADPH/NADP+ gradient high
Isoprenoid and sterol synthesis early stages
FA syntehsis

Question 8

How do FA move in blood

Answer 8

on albumin

Question 9

Hormone sesntive lipase

Answer 9

@ glucagon binding resulting in cAMP/PKA activation

• makes cleave TAGs into monoglycerols then to FA
• as fatty acids and albumin to muscle where beta ox can occur

or at epi (same result)

Question 10

FA syntehsis results in

Answer 10

form activated acyl group and activated malonyl gorup to activvated acyl group that is bigger by two carbons

creates and FADH2 and NADH

goes into TCA and makes 3 NADH, 1 FADH2, and 1 GTP

Question 11

Carnitine palmitoyl transferase

Answer 11

CPT1 and CAT1 are same
-also carnitine acyl transferase

Makes FA coa to acetyl carnitine
(long ones-short/medium ones can diffuse_

Question 12

carnitine-acylcarnitine translocase

Answer 12

CAT/CACT

acylcarnitine into mito matrix for carnitine out

Question 13

CPT2

Answer 13

same as CAT2

acyl carnitine to FA COA

Question 14

Unsaturated FA oxidation

Answer 14

yields less FADH2 beucase partially oxidized

Question 15

alpa oxidation

Answer 15

oxidation of branched chain FA to acetyl coa and propionyl CoA
-from plant chlorophylls

Question 16

MCAD

Answer 16

although Medium chain FA can enter mito matrix without shuttle-need MCAD for oxidation

Question 17

Adrenoleukodystrophy
aka
Def
inheritance
Sx

Answer 17

ALD

Def at ALDP protein not allowing long FA acids to go into peroxisome

X linked

dysfunction of adrenal cortex and demyelination, High levels of saturated long FA chains in tissues sand flluids

Question 18

Insulin on TAG syn

Answer 18

increase glucose to DHAP

Question 19

Ethonol on TAG syn

Answer 19

VLDL cant leave liver-fatty liver

Question 20

glucagon and cortisol and epi on TAG syn

Answer 20

increase breakdown of TAG to glycerol and FA

increase glycerol to glucose (gluconeogenesis)-not epi

Question 21

Carnitine shuttlle deffect

• types
• whats defected
• sx
• trx

Answer 21

Primary-CPT1 or 2 def
Secondary-low dietary carnitine

Sx-muscle pain/fatigue after excerisem incrase FA in blood, ypoketotic hypoglycemia

High carb diet with short and medim chain FA

Question 22

CPT1 def

• rarity
• what is primarilty effected
• what occurs
• sx

Answer 22

rare
primary effects liver
Less FA ox and ketogenesis
Hypoketotc hypoglyemia
elevation of carnitine in blood
hepatomegaly + muscle weakess

Question 23

CPTII def

3 from

Answer 23

Adult myopahtic
-SM, caues muscle pain, fatigue m, and myoglobin uria

Severe infantile multisystem

• hypoketotic hypoglycemia
• hepato/cardiomegaly

Rareest no nata form

• 4 hours after birth
• resp failure
• cardiomegaly, arryhtmias, hepttomegaly, seizures

Question 24

MCAD def
-what
-sx
trx
similar to?

Answer 24

Impaired ox of medium chain FA

Hypoketotic hypoglyemia
-no enough acetyl CoA

dicarboxylic acids due to omega ox, and other carboxlyic acids

hyperaommina-dliver damage

cant metaboize fats during fasting-limits gluconeogenesis

frquent feeding and carnitine sup

similar to short/long chain dehydrogenase def

Question 25

why is carnitine trx for MCAD def

Answer 25

FA into mito-builds up cuz no MCAD

• goes to CPT2 to get carnitine to go back to circulatoin
• –steals carnitines from short and long FA

Question 26

where are ketone formed vs cholesterol

Answer 26

mito vs cyto/er

• in liver
• 1st/second reactions same

Question 27

CoA transferase

Answer 27

Thiophorase
Acetoacetate (from 3 hydroxybutyrate) to succinate=replenlsh TCA cycle

NOT IN LIVER-LIVER CAN NOT USE KETONE BODIES

Question 28

Thiolase

Answer 28

Acetyl CoA and CoA to 2 aceryl CoA=oxaloacetate=TCA cycle

Question 29

@ increase FA

Answer 29

hormone senstive lipase is active

• lots of Beta ox=lots of FA
• malonyl CoA low-beta ox can occur
• more Acetyl CoA-lots of NADH-TCA cycle reversis and oxaloacete makes malate for gluconeogenesis

@accumulation of acetl coa=acetoacyl coa=ketones

Question 30

2 things needed for ketone body formation

Answer 30

build up of acetyl coa and slow down/reversion of TCA

Question 31

@ fasting/diabetic what happens to TAG and ketone bodies

Answer 31

When more glucagon vs insulin
Degrade TAG-release glycerol and FA
-FA to acetyl CoA-build up-ketone bodies form
-Glycerol to gluose
-Lots of acetyl CoA (from 3 hydroxybutyrate to acetoacetyl coa)-into TCA

Question 32

Heart prefers vs brain

Answer 32

heart likes acetoacetate (from3 hydroxybutrate)

Brain likes glucose but can use acetoacetate during starvation and diabetes

Question 33

FA, acetoacetate, and beta hydroxybutyrate @ normal ,fasting, starvation ocnidtions

Answer 33

increase with each condition

Question 34

DM and ketone bodies

Answer 34

cuz glucose not taken up by liver, cant make OAA

• TCA slows and when FA released from adipose cant go into TCA
• so makes ketone bodies

Question 35

Hypoketotoic hypoglycemia etiology

Answer 35

No carnitine transporter
-no way to FA to get into cell/mito

No ketone bodies produced from FA=hypoglycemia