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Defective cholsterol ABC transporter, low apo A-1, HDL, leads to hypolipidemia
Tangier disease
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similar to LDL but has apo a bound to apo B-100
Lp(a)
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three risk factors for heart disease
oxLDL, LDL pattern B, Lp(a)
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hypercholesterolemia can lead to this : cholesterol rich material in tendons
xanthomas
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ID Hperlidemia type? LPL deficiency, high CM
type I (rare)
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ID hyperlipidmia type: LPL deficiency, high level of VLDL
IV (common)
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ID Hyperlipidemia type: LDL receptor deficiency, lead to high LDL, treat with statins and bile acid sequestrants
IIa (common)
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High LDL and VLDL. Due to LDL receptor deficiency or overproduction of VLDL. Treat with NIACIN, statins, or fibrates.
IIb (common)
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ID hyperlipidemia type: apoE deficiency, high IDL and high VLDL,
III (rare)
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Contents in chylomicron remnants. Where is it going to?
has CE, lipid soluble vit, essential FA, TAG
to the liver
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Two sources of PC
- diet
- methylation of PE (3X with SAM)
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deficiency leads to increased DHEA but no gluco mineralocorticoid production
3 B hydroxysteroid DH
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deficiency of this enzyme leads to: less cortisol and less DHEA production. over production of aldosterone. Female like genitalia.
CYP 17 deficiency
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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
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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)
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Cox 2 specific inhibitor drug? why can't it inhibit COX1?
celecoxib (larger and cannot enter cox1)
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How does aspirin inhibit COX (molecular)
aspirin irreversibly acetylate the serine residue at the active site
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This leukotriene increases vascular permeability and act as chemoattractant for neutrophils
LTB
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this leukotriene has tripeptide GSH and increase vascular permeability, lead to broncoconstriction
LTC
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these leukotrienes are part of SRS-A
cystenyl leukotrienes (LTC, LTD, LTE)
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LPL and Apo CII is needed for what action in VLDL
TAG cleavage in VLDL
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cleaves TAGs in IDLs to form LDL
HTGL ( hepatic lipase)
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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
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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)
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brain crossing pattern of FA and cholesterol
FA can cross BBB but cholesterol can't (cholesterol must be synthesizd inside the brain)
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treatment for gallstone formation
give chenodeoxycholic acid
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LPL synthesis depends which hormone?
insulin
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which LPL is smaller? heart LPL or tissue LPL
heart LPL
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Smallest lipoprotein, highest % of protein, apo C-11, apo E, apo A-1
HDL
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these two apoproteins are needed for taking the chylomicron remnant to the liver
ApoE and Apo B48
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what happens to cholesterol esters taken up into liver by lipoprotein
the are cleaved to free cholesterol
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this enzyme : cholesterole-----> bile
7 alpha hydroxylase
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two main functions of HDL
1) provide apoC-II and apoE to chylomicron and VLDL
2) reverse cholesterol transport to the liver
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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.
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in blood, this enzyme makes CE from free cholesterol
LCAT or PCAT
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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
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what happens to the CE formed by LCAT
goes directly into HDL forming round shaped HDL3 (entrapment)
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role of CETP (cholesterol ester transfer protein)
lets HDL3 release CE to VLDL and takes up phospholipid and TAG from VLDL. (HDL3--->HDL2 (larger))
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which one is larger HDL3 or HDL2
HDL2
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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)
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LRP? whats it for? what apoproteins does it recognize?
Receptor in Liver for reuptake of IDL. It looks for ApoE and Apo100 on IDL.
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this enzyme is needed for producing cytosolic Acetyl CoA
citrate lyase
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malonyl coA inhibits
CPT1
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de novo FA synthesis creates this molecule
palmitate
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where does elongation, desaturation of FA, TAG synthesis accur
ER-----------once FA (palmitate) is synthesized in the cytosol, these further processes occur)
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measure this to look for essential FA deficiency (EFA deficiency)
mead acid
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substrates needed for acetyl CoA carboxylase
for FA synthesis---CO2, ATP, biotin, and acetyl CoA (will need to be activated later)
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what is released after palmitate formation? how many NADPH required for 2C elongation?
CO2 used was released, 2NADPH
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generation of NADPH uses which enzyme and PPP?
malic enzyme
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low NADPH activates which enzyme in the PPP, its function
G6P dehydrogenase, help controle high blood glucose level
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malate. NADP+ with malic enzyme generates
pyruvate, NADPH, CO2
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