Fatty Acid and TAG Synthesis Flashcards
linoleic acid
linolenic acid
arachidonate
omega 6-pro inflammatroy-SMASH
omega 3-anti-inflammatroy
synthesized from linoloic-omega 6-prostaglanding precursor
why cant mammals syntehsize omega 6 or 3
dont have enzyme to introduce double bonds beyond C9 in FA
Essential FA def
dry/flaky/scaly/lackluster skin, small bumps, mixed oily and dry skin
where are FA synthesized
liver, lactating tissue
-and lesser exxtent adipose
what occurs in mito
FA ox, acetyl Coa production, ketone body syn, FA elonation
wha toccurs in ER
phospholipid synthesis
late stage sterol syn
FA elongation
FA desaturation
what occurs in cytosol
NADPH production-HMP/malic enzyme
NADPH/NADP+ gradient high
Isoprenoid and sterol synthesis early stages
FA syntehsis
How do FA move in blood
on albumin
Hormone sesntive lipase
@ 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)
FA syntehsis results in
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
Carnitine palmitoyl transferase
CPT1 and CAT1 are same
-also carnitine acyl transferase
Makes FA coa to acetyl carnitine
(long ones-short/medium ones can diffuse_
carnitine-acylcarnitine translocase
CAT/CACT
acylcarnitine into mito matrix for carnitine out
CPT2
same as CAT2
acyl carnitine to FA COA
Unsaturated FA oxidation
yields less FADH2 beucase partially oxidized
alpa oxidation
oxidation of branched chain FA to acetyl coa and propionyl CoA
-from plant chlorophylls
MCAD
although Medium chain FA can enter mito matrix without shuttle-need MCAD for oxidation
Adrenoleukodystrophy aka Def inheritance Sx
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
Insulin on TAG syn
increase glucose to DHAP
Ethonol on TAG syn
VLDL cant leave liver-fatty liver
glucagon and cortisol and epi on TAG syn
increase breakdown of TAG to glycerol and FA
increase glycerol to glucose (gluconeogenesis)-not epi
Carnitine shuttlle deffect
- types
- whats defected
- sx
- trx
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
CPT1 def
- rarity
- what is primarilty effected
- what occurs
- sx
rare primary effects liver Less FA ox and ketogenesis Hypoketotc hypoglyemia elevation of carnitine in blood hepatomegaly + muscle weakess
CPTII def
3 from
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
MCAD def -what -sx trx similar to?
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
why is carnitine trx for MCAD def
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
where are ketone formed vs cholesterol
mito vs cyto/er
- in liver
- 1st/second reactions same
CoA transferase
Thiophorase Acetoacetate (from 3 hydroxybutyrate) to succinate=replenlsh TCA cycle
NOT IN LIVER-LIVER CAN NOT USE KETONE BODIES
Thiolase
Acetyl CoA and CoA to 2 aceryl CoA=oxaloacetate=TCA cycle
@ increase FA
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
2 things needed for ketone body formation
build up of acetyl coa and slow down/reversion of TCA
@ fasting/diabetic what happens to TAG and ketone bodies
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
Heart prefers vs brain
heart likes acetoacetate (from3 hydroxybutrate)
Brain likes glucose but can use acetoacetate during starvation and diabetes
FA, acetoacetate, and beta hydroxybutyrate @ normal ,fasting, starvation ocnidtions
increase with each condition
DM and ketone bodies
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
Hypoketotoic hypoglycemia etiology
No carnitine transporter
-no way to FA to get into cell/mito
No ketone bodies produced from FA=hypoglycemia
