Defective cholsterol ABC transporter, low apo A-1, HDL, leads to hypolipidemia
similar to LDL but has apo a bound to apo B-100
three risk factors for heart disease
oxLDL, LDL pattern B, Lp(a)
hypercholesterolemia can lead to this : cholesterol rich material in tendons
ID Hperlidemia type? LPL deficiency, high CM
type I (rare)
ID hyperlipidmia type: LPL deficiency, high level of VLDL
ID Hyperlipidemia type: LDL receptor deficiency, lead to high LDL, treat with statins and bile acid sequestrants
High LDL and VLDL. Due to LDL receptor deficiency or overproduction of VLDL. Treat with NIACIN, statins, or fibrates.
ID hyperlipidemia type: apoE deficiency, high IDL and high VLDL,
Contents in chylomicron remnants. Where is it going to?
has CE, lipid soluble vit, essential FA, TAG
to the liver
Two sources of PC
methylation of PE (3X with SAM)
deficiency leads to increased DHEA but no gluco mineralocorticoid production
3 B hydroxysteroid DH
deficiency of this enzyme leads to: less cortisol and less DHEA production. over production of aldosterone. Female like genitalia.
CYP 17 deficiency
the effect of CYP 21 or CYP11B deficiency on cortisol , DHEA, and testosterone synthesis( specifically prenatal/postnatal)
less cortisol more DHEA, females have prenatal masculinization and males will have postnatal virilization
the effect of CYP 21 and 11B deficiency on aldosterone production
CYP 21 deficiency leads to HYPOtension ( b/c no deoxycorticosterone nor aldosterone produced). CYP11B deficiency will yield no aldosterone but deoxy corticosterone produced ( HYPERtension due to deoxy corticosterone production)
Cox 2 specific inhibitor drug? why can't it inhibit COX1?
celecoxib (larger and cannot enter cox1)
How does aspirin inhibit COX (molecular)
aspirin irreversibly acetylate the serine residue at the active site
This leukotriene increases vascular permeability and act as chemoattractant for neutrophils
this leukotriene has tripeptide GSH and increase vascular permeability, lead to broncoconstriction
these leukotrienes are part of SRS-A
cystenyl leukotrienes (LTC, LTD, LTE)
LPL and Apo CII is needed for what action in VLDL
TAG cleavage in VLDL
cleaves TAGs in IDLs to form LDL
HTGL ( hepatic lipase)
How much of LDL is taken up by the liver? where else does it go to?
70%, the rest goes to cells that need cholesterol or the cells with LDL receptors
genetic defect in cholesterol synthesis, microencephaly, low IQ. this is defieciency in what enzyme?
SLOS (smith lemili opitz), defic. 7-dehydrocholesterol reductase (no ring B formation)
brain crossing pattern of FA and cholesterol
FA can cross BBB but cholesterol can't (cholesterol must be synthesizd inside the brain)
treatment for gallstone formation
give chenodeoxycholic acid
LPL synthesis depends which hormone?
which LPL is smaller? heart LPL or tissue LPL
Smallest lipoprotein, highest % of protein, apo C-11, apo E, apo A-1
these two apoproteins are needed for taking the chylomicron remnant to the liver
ApoE and Apo B48
what happens to cholesterol esters taken up into liver by lipoprotein
the are cleaved to free cholesterol
this enzyme : cholesterole-----> bile
7 alpha hydroxylase
two main functions of HDL
1) provide apoC-II and apoE to chylomicron and VLDL
2) reverse cholesterol transport to the liver
shape and the composition of HDL ? why this shape?
HDL is discoidal shaped ( empty phospholipid, for reuptake of CE). It is made with PC (phospholipid) with apo A-1.
in blood, this enzyme makes CE from free cholesterol
LCAT or PCAT
where is LCAT syntehsized? what from HDL is required for activation? whats the substrate for LCAT's rxn
Liver, Apo A-1, use PC from the mambrane and the free cholesterol
what happens to the CE formed by LCAT
goes directly into HDL forming round shaped HDL3 (entrapment)
role of CETP (cholesterol ester transfer protein)
lets HDL3 release CE to VLDL and takes up phospholipid and TAG from VLDL. (HDL3--->HDL2 (larger))
which one is larger HDL3 or HDL2
receptor and the enzyme used in reuptake of CE to the liver cells? what happens to the HDL then?
SR -B1, hepatic lipase, HDL2 turns back to HDL3 (smaller)
also happens is the phospholipid cleavage of HDL2 (becasue HTGL cleaves TAG and phospholipid)
LRP? whats it for? what apoproteins does it recognize?
Receptor in Liver for reuptake of IDL. It looks for ApoE and Apo100 on IDL.
this enzyme is needed for producing cytosolic Acetyl CoA
malonyl coA inhibits
de novo FA synthesis creates this molecule
where does elongation, desaturation of FA, TAG synthesis accur
ER-----------once FA (palmitate) is synthesized in the cytosol, these further processes occur)
measure this to look for essential FA deficiency (EFA deficiency)
substrates needed for acetyl CoA carboxylase
for FA synthesis---CO2, ATP, biotin, and acetyl CoA (will need to be activated later)
what is released after palmitate formation? how many NADPH required for 2C elongation?
CO2 used was released, 2NADPH
generation of NADPH uses which enzyme and PPP?
low NADPH activates which enzyme in the PPP, its function
G6P dehydrogenase, help controle high blood glucose level