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26 Cards in this Set

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FA synthesis
use the SYtrate shuttle

Acetyl-CoA ----ACC--> malonyl-CoA --> FA synthesis

***Rate limiting: Acetyl CoA Carboxylase
FA degradation
CARnage of FA
FA + CoA ---> (with FA CoA synthetase) Acyl-CoA ----> (with acyl-CoA DH) beta-oxidation to acetyl-CoA for ketone bodies and TCA

rate-limiting: carnitine acyltransferase (Carnitine shuttle)
Carnitine deficiency
can't transport LCFAs into mitochondria -->toxic accum

weakness, hypotonia, hypoketotic hypoglycemia
Acyl-CoA DH def
increase dicarboxylic acids, decrease glucose and ketones
Ketones
In liver, FA and AA are metabolized into:
Acetoacetate (some will convert to acetone)
Beta-hydroxybutyrate (used in muscle and brain)

Starvation: oxaloacetate depleted for GNG
Alcoholism: excess NADH shunts oxaloacetate to malate

result: TCA stalled --> shunt glucose and FFA toward ketogenesis

rate: HMG-CoA synthase
Fed state
glycolysis and aerobic respiration
Fasting
Hepatic glycogenolysis
hepatic GNG
adipose release of FFA
Starvation 1-3days
1. Hepatic glycogenolysis (depleted after 1 day)
2. Adipose release of FFA
3. Muscle and liver, shift fuel use from glucose to FFA
4. Hepatic GNG (lactate, alanine, adipose glycerol & propionyl-CoA (OCFA)
Starvation >3days
Adipose stores: ketones main energy for brain and heart

after depleted: vital protein degradation --> organ failure and eath
Lecithin-cholesterol acyltransferase (LCAT)
catalyzes esterification of cholesterol (2/3)

ex: nascent HDL --> Mature HDL
Cholesterol ester transfer protein (CETP)
mediates transfer of cholesterol esters to other lipoprotein particles

-Allows HDL to deposit chol onto VLDL, IDL, LDL
A-I
Activates lcAt, which catalyzes esterification of cholesterol (nascent HDL --> mature HDL)
B-100
Binds to LDL receptor, mediates VLDL secretion
C-II
C-LL
Cofactor for LPL
B-48
Mediates chylomicron secretion
E
Mediates Extra (remnant) uptake
C-III
inhibits LPL
LDL
transports chol from liver to tissues:
formed by LPL modification of VLDL in peripheral tissue
-taken up by target cells via receptor-mediated endocytosis

apolipoproteins: B-100
HDL
transports chol from periphery to liver
-repository for apoC and apoE (needed for chylomicron and VLDL metabolism
-secreted from liver and intestine
Chylomicron
-delivers dietary TGs to peripheral tissue
-delivers chol to liver in the form of chylomicron remnants (mostly depleted of their triacylglycerols)
-secreted by intestinal epithelial cells

apolipoproteins:
B-48, A-IV, C-II, E
VLDL
delivers hepatic TGs to peripheral tissue; secreted by liver

apolipoproteins:
B-100, C-II, E
IDL
formed in degardation of VLDL
-delivers TGs and chol to liver, where they are degraded to LDL

apolipoproteins: B-100, E
Type I: hyperchylomicronemia
Increased chylomicrons

elevated TG, chol

LPL def or altered apo C-II (cofactor for LPL)

causes pancreatitis, HSM, eruptive/pruritic xanthomas (no increased risk for atherosclerosis)
Type IIa: familial hypercholesterolemia
Auto dom
increased LDL

elevated cholesterol

**absent or decreased LDL receptors

causes accel atherosclerosis, tendon xanthomas, corneal arcus
Type IV: hypertriglyceridemia
increased VLDL

elevated TG

due to hepatic overproduction of VLDL
causes pancreatitis
Abetalipoproteinemia
inability to synthesie lipoproteins due to def in apoB-100 and apoB-48

sxs in first few months of life; FTT, steatorrhea, acanthocytosis (vit E), ataxia (vit E), night blindness (vit A)

bx shows accum of lipids within enterocytes; can't secrete chylomicrons

Tx: vit E (bc of def and helps restore lipoproteins); TG restriction