Term
|
Definition
| Complexs I, II, III; non-heme iron complexed to sulfur of a cysteinyl side chain |
|
|
Term
|
Definition
| Contain Fe-prophyrin group (Fe = heme) |
|
|
Term
|
Definition
|
|
Term
| NADH-Q reductase complex I (NADH dehydrogenase/NAD reductase) |
|
Definition
| Proton pump; contains FMN, Fe-S centers (e- from NADH --> FMN --> Fe-s --> CoQ |
|
|
Term
| Cytochrome C reductase complex III (cytochrome reductase) |
|
Definition
| Proton pump; contains cytochrome C, cytochrome B and Fe-S center |
|
|
Term
| Cytochrome oxidase complex IV |
|
Definition
| Proton pump; O2 clamped between heme a3 and Cu during reduction to H2O |
|
|
Term
| Succinate-FAD dehydrogenase complex II |
|
Definition
| ROS production = decreased ATP production; heme B is not in direct path of e- transfer |
|
|
Term
|
Definition
| Binding of ADP --> phosphorylation --> release of ATP (aresenic competes for Pi site); reversibility allows ATP dependent membrane transport of H+ into lysosome |
|
|
Term
| Control of oxidative phosphorylation |
|
Definition
| Dependent on availability of ADP |
|
|
Term
|
Definition
| Inhibits e- flow from Fe-S to ubiquinone |
|
|
Term
|
Definition
| Inhibits e- flow through NADH-Q complex |
|
|
Term
|
Definition
| Inhibits e- flow through QH2-cytochrome C reductase complex |
|
|
Term
|
Definition
| Lethal; blocks ETC by binding Fe3+ in cytochrome a/a3 |
|
|
Term
|
Definition
|
|
Term
|
Definition
| Outcompetes Pi on transport and Pi site on ATP synthase and phsophoglycerate kinase in glycolysis |
|
|
Term
| Pentachlorophenol & 2,4-dinitrophenol |
|
Definition
| Dissapates H+ gradient without running ATP synthase |
|
|
Term
|
Definition
| Deposits form at basal ganglia --> uncoupling of oxidative phosphorylation = brain damage |
|
|
Term
| Thermogenin (in brown adipose) |
|
Definition
| Allows H+ to pass down gradient without ATP production (thermal control of critical tissues) |
|
|
Term
| Leber's Hereditary Optic Neuropathy |
|
Definition
| Missense mutation in complex I --> no e- transfer from NADH -->CoQ = decreased ATP yield |
|
|
Term
| Most common cause of oxidative phosphorylation failure |
|
Definition
|
|
Term
| Pentose phosphate pathway major regulatory step |
|
Definition
| Oxidative sequence; G6PD allosteric inhibition by NADPH (6-phosphogluconate dehydrogenase is also inhibited by NADPH) |
|
|
Term
| Transketolase requires? Transfers? |
|
Definition
| TPP bound to Schiff base; transfers 2C keto fragment to aldose phosphate |
|
|
Term
|
Definition
| Transferse 3C fragment to aldose acceptor |
|
|
Term
| Wernicke-Korsakoff Syndrome |
|
Definition
| Transketolase defect that reduces afinity got vitamin B1 --> normal, equilibrium reaction turns into a rate limiting step |
|
|
Term
| Ribonucleotide reductase maintianed by: |
|
Definition
| Maintained in proper state by thioredoxin reductase (require NADPH) |
|
|
Term
| Glutathione and folate derivatives are reduced by: |
|
Definition
|
|
Term
| Glutathione (amino acids) |
|
Definition
| glu-cys-gly; SH groups keep critical cytosine residues in essential reduced state; central to defense against oxidative toxicity |
|
|
Term
|
Definition
| Denatured Hb due to -SH oxidation and loss of tertiary RBC structure |
|
|
Term
|
Definition
| Requires selenium (Se) as cofactor; reduces organix peroxides and alcohols; reverses lipid peroxidation |
|
|
Term
|
Definition
| Oxidized by glutathione peroxidase, then 2nd reductase re-established -SH groups |
|
|
Term
| Glutathione-S-transferase |
|
Definition
| Attaches glutathione to toxic molecules, making them more soluble |
|
|
Term
| Detoxification of acetaminophen |
|
Definition
| 1. Acetominophen broken down by CYP450 into NAPQI 2. NAPQI broken into a soluble, non-toxic substance to be excreted via glutathione |
|
|
Term
| CYP450 enzymes; induced by: |
|
Definition
| NADPH dependent mono-oxygenases; induced by phenobarbital, barbiturates, griseofulvin |
|
|
Term
|
Definition
| Ca2+ dependent (L-argining+O2+NADPH-->Citrulline+NO+NADP |
|
|
Term
| Guanylate phosphodiseterase |
|
Definition
| cGMP --> 5'GMP; inhibited by viagra |
|
|
Term
|
Definition
| e- combined with NO --> OONO-'; can cause peroxidation and nitration of tyrosyl hydroxide in proteins |
|
|
Term
| Amyotrophic lateral sclerosis (ALS) |
|
Definition
| Cytosolic superoxide dismutase defect |
|
|
Term
| Cytosolic superoxide dismutase |
|
Definition
|
|
Term
| Mitochondrial superoxide dismutase |
|
Definition
|
|
Term
|
Definition
| Contains heme and Fe2+; breaks down 1/4 total alcohol (remainder by alcohol dehydrogenase) |
|
|
Term
|
Definition
| Rapid glucose metabolism by pentose phosphate pathway to produce NADPH; HOCl (hypochlorous acid) |
|
|
Term
|
Definition
|
|
Term
| Hereditary fructose intolerance ('fructose poisoning') |
|
Definition
| Aldolase B deficiency --> increased F1P --> lacticacidemia, hyperuricemia --> liver failure, death |
|
|
Term
|
Definition
| Universal sugar activiting group always added to C1 |
|
|
Term
|
Definition
|
|
Term
|
Definition
| Linked to threonine, serine; glycocalyx |
|
|
Term
|
Definition
| Activates sialic acid (NANA) |
|
|
Term
|
Definition
| Oligosaccharide carrier in ER |
|
|
Term
|
Definition
| NADPH reduction makes it more difficult for the compound to cross the membrane (active in specialized tissues) |
|
|
Term
|
Definition
| Uridyl transferase deficiency --> accumulation of galactose-1-P and galactitol |
|
|
Term
|
Definition
| Inhibits alpha-lactalbumin synthesis |
|
|
Term
|
Definition
| Stimulates alpha-lactalbumin |
|
|
Term
| UDP-galactose:glucose galactosyltransferase (lactose synthase) |
|
Definition
| Composed of galactosyltransferase and alpha-lactalbumin |
|
|
Term
|
Definition
| Active = dephosphorylated, polymer; inactive = phosphorylated (monomer); reuqires biotin bound to Schiff base as CO2 carrier; Activated by citrate, insulin; inhibited by malonyl/palmitoyl CoA, glucagon, Epi |
|
|
Term
|
Definition
| Covalently bound to monomer of fatty acid synthase |
|
|
Term
| Acyl Carrier Protein (domain of fatty acid synthase) |
|
Definition
| Pantothenic acid; -SH group creates thioester bond with acyl units |
|
|
Term
|
Definition
| Acetyl-CoA carboxylase (acetyl-CoA --> malonyl-CoA), ATP citrate lyase (citrate-->OAA + acetyl-CoA); G6P6; FAS |
|
|
Term
| Desaturases (ER bound desaturase system) |
|
Definition
| Mono-oxygenases; reuqire O2 + NADH/NADPH to produce 2H2O; cytochrome b5 channels e- from NAD(P)H to 1/2O2 |
|
|
Term
|
Definition
| Leads to lipofuscin accumulation ('age-pigment') |
|
|
Term
| Release of fatty acids from hydrophobic energy stores |
|
Definition
| Phosphorylation of perilipin / TAG cleavage by hormone-sensitive lipases |
|
|
Term
|
Definition
| 1x lysine, 3x methionine via S-adenosyl-methionine; shuttles LCFAs into matrixl; note that FAs < 12C do not require carnitine, but all Fas must be activated with CoASH |
|
|
Term
| R/Q (respiratory quotient) |
|
Definition
|
|
Term
| Primary regulation of B-oxidation (enzymes?) |
|
Definition
| Hormone sensitive lipase, lipoprotein lipase |
|
|
Term
| Secondary regulation of B-oxidation |
|
Definition
| Malonyl-CoA inhibits carnitine-acyl transferase I in the innermembrane space (no regulation of carnitine-acyl transferase II) |
|
|
Term
|
Definition
| No functioning peroxisomes = decreased plasmalogen, ether lipid syntheses |
|
|
Term
|
Definition
| Branched chain Fas --> phytanic acid |
|
|
Term
|
Definition
Alpha carbon cannot be hydroxylated (alpha-hydroxylase deficiency) --> accumulation of phytanic acid
B-oxidation cannot proceed as propionyl-CoA is not produced = increase in VLCFAs and decrease in plasmalogens |
|
|
Term
| Propionyl-CoA carboxylase requires: |
|
Definition
|
|
Term
|
Definition
|
|
Term
| Medium chain acyl-CoA dehydrogenase deficiency |
|
Definition
| Fas > 6C cannot be catabolized to acetyl-CoA |
|
|
Term
| Vitamin B20 deficiency due to? |
|
Definition
| Commonly due to acyl-CoA dehydrogenase deficiency |
|
|
Term
| Ketogenesis occurs where? Tissues that don’t use ketones? Enzyme deficiency? |
|
Definition
Occurs in mitochondria of liver only
All tissues use ketones except for liver, RBC (lack acetoacetate:succinyl-CoA:CoA transferase AKA ketoacid transferase) |
|
|
Term
|
Definition
Prostaglandins, thromboxanes, leukotrienes
Made in all cells except RBCs and lymphocytes |
|
|
Term
| Primary source of arachidonic acid |
|
Definition
| Phosphatidylinositol via phospholipase A2 cleavage |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Produced by vscular endothelium; 5 membered ring |
|
|
Term
|
Definition
| Produced by platelets; 6 membered ring |
|
|
Term
|
Definition
|
|
Term
|
Definition
| Inducible (response to inflammation); suppressed by glucocoritcoids |
|
|
Term
|
Definition
| Potent vasodilators via cAMP increase; exception: induce uterine contractions in endometrium |
|
|
Term
|
Definition
| Vasoconstriction via cAMP decrease |
|
|
Term
|
Definition
Power constrictors of smooth muscle
Glutathione is a requires component; increase in SRS-A (slow-reacting substance of anaphylaxis) |
|
|
Term
|
Definition
| Glucocorticoids: blocks PLA2 = blocks all production |
|
|
Term
|
Definition
| Aspirin, indomethicin, ibuprofen = inhibits COX only-->prevents production of TXA2 |
|
|
Term
|
Definition
| Ceramide + phosphocholine; only phospholipid not containing glycerol |
|
|
Term
|
Definition
|
|
Term
|
Definition
| Unsaturated FA at C1 with ether link (PA-ethanolamine in nervous tissue, PA-choline in heart) |
|
|
Term
| Platelet activating factor |
|
Definition
| Saturated FA at C1 with ether link + acetyl group at C2; triggers thrombotic reactions |
|
|
Term
| Dipalmitoylphosphatidylcholine |
|
Definition
| Key component of lung surfactant; palmitoyl chains on C1, C2; activation via CTP-phosphocholine-cytidylyltransferase |
|
|
Term
| Phosphatidyl-4,5-bisphosphate |
|
Definition
| Stearate on C1, arachidonate on C2 = reservior for ecosanoids |
|
|
Term
|
Definition
Acts on phosphatidylinositol, phosphatidylcholine to release arachidonic acid
Activated by trypson, inhibited by glucocorticoids |
|
|
Term
|
Definition
| In liver lysosomes; activated by PIP2 to produce second messengers |
|
|
Term
|
Definition
| Lysosomal phospholipase that degrades sphingomyelin: removal of phosphorylcholine, leaving ceramide |
|
|
Term
|
Definition
| Releases sphingosine and FA |
|
|
Term
|
Definition
| Lack of PLC, sphingomyelinase; type A = neurodegeneration, type B = no neural involvement |
|
|
Term
| Asymmetry of phospholipids: outer leaflet |
|
Definition
| Sphingomyelin, phosphaditylinositol |
|
|
Term
| Asymmetry of phospholipids: inner leaflet |
|
Definition
| Phosphatidylserine, phosphatidylethanolamine, phsophatidylcholine |
|
|
Term
|
Definition
| Neutral glycosphingolipids; ceramide + glucose/galactose via O-glycosidic bond |
|
|
Term
|
Definition
| Acidic glycosphingolipids; NANA - requires CMP activation, always on terminal posistion in oligosaccharide chain |
|
|
Term
| PAPS (3'-phosphoadenosine-5'-phosphosulfate) |
|
Definition
| Donates sulfate (via sulfotransferasesP to sulfatides (sulfated galactocerebrosides) |
|
|
Term
|
Definition
| Repeating disaccharide units made of acidic sugar (glucuronic/iduronic acid) and/or amino sugar (N-acetyl-glucosamine) |
|
|
Term
| Protein core of all GAGs except hyaluronic acid |
|
Definition
|
|
Term
|
Definition
| Most abundant; cartilage, tendons, ligaments, aorta |
|
|
Term
|
Definition
| Galactose, N-acetlyglucosamine; cartilage, cornea of eye |
|
|
Term
|
Definition
| L-iduronic acid, N-acetylglucosamine; skin, heart valves, blood vessels |
|
|
Term
|
Definition
| Glucuronnic acid, N-acetylglucosamine; loose CT, cynovial fluid, vitreous humor |
|
|
Term
|
Definition
| Intracellular; iduronic/glucuronic/glucosamine residues |
|
|
Term
|
Definition
| Extracellular; basement membrane; promotes neural outgrowth (inhibited by chondroitin-sulfate) and oligomerization of receptor protein tryosine kinase |
|
|
Term
| Anchor for xylose, galactose |
|
Definition
|
|
Term
|
Definition
| Serine-xylose-galactose-galactose; driven by UDP activation |
|
|
Term
|
Definition
| alpha-L-iduronidase; corneal clouding |
|
|
Term
|
Definition
| Iduronate sulfatase; no corneal clouding (x-linked) |
|
|
Term
| Signal for secreted globular proteins |
|
Definition
| Hydrophobic signal peptide |
|
|
Term
| Membrane protein topology: type I |
|
Definition
| N-term signal sequence cleaved; C-term = cytoplasmic |
|
|
Term
| Membrane protein topology: type II |
|
Definition
| Signal sequence not cleaved; N-term = cytoplasmic |
|
|
Term
| Membrane protein topology: type III |
|
Definition
| Signal sequence not cleaved; C-term = cytoplasmic |
|
|
Term
|
Definition
| Beta-glucoronidase; affects degradation of dermaten, heparin sulfate |
|
|
Term
| Asparagine linked (N-linked) signal |
|
Definition
| Asn-X-Serine/Threonine; for high mannose content class; second class = complex oligosaccharides |
|
|
Term
|
Definition
| A-antigen: N-acetylglucosamine; B-antigen: galactose |
|
|
Term
|
Definition
| Cleaves protein in constitutive secretion pathway (proalbumin) |
|
|
Term
|
Definition
| Cleaves protein in regulated secretion pathway (proinsulin) |
|
|
Term
| Protein disulfide isomerase |
|
Definition
| Promotes new disulfide bond formation in ER until most stable conformation is reached |
|
|
Term
|
Definition
| Reversable (competitive) inhibitor of HMG-CoA reductase |
|
|
Term
|
Definition
| Moves 15C unit to CAAX motif of protein; target for inhibition in cancer chrmotherapy |
|
|
Term
|
Definition
| Ubiquinone, dolichol, cytochrome a/a3 prosthetic group, farnesyl/geranyl groups |
|
|
Term
|
Definition
| Prodices malformation of myelin in Refsum's disease due to alpha-hydroxylase deficiency |
|
|
Term
|
Definition
| Cholic and chenodeoxycholic acid; can be conjugated with glycine/taurine (3:1) to reduce pH |
|
|
Term
| Control of bile acid synthesis |
|
Definition
| HMG-CoA reducatase, NFB at 7-alpha-hydroxylase (regulated step) |
|
|
Term
| 7-alpha-hydroxylase; requires? |
|
Definition
| Catalyzes regulated step of bile acid synthesis; requires O2, NADPH, CYP450, Ascorbic acid |
|
|
Term
| Bile salt enterohepatic vasculature |
|
Definition
| Primary bile salts made by liver --> deconjugation of taurine/glycine, cholic acid and removal of 7-alpha-hydroxylase to make neutral sterols (secondary bile salts) --> resorbtion |
|
|
Term
| Vitamin B12 requiring enzymes |
|
Definition
| Methylmalonyl-CoA mutase, homocysteine methyltransferase |
|
|
Term
| Corticosteroids, progestins side chain at C17 |
|
Definition
|
|
Term
| Androgens, estrogens side chain at C17 |
|
Definition
|
|
Term
| Mineralocorticoid group at C18 |
|
Definition
|
|
Term
| Steroidogenic acute regulatory protein (StAR) |
|
Definition
Activated by PKA
Regulates cholesterol transfer into mitochondria
Rate limiting step in production of steroid hormones |
|
|
Term
|
Definition
| Mineralocorticoid production (contains aldosterone synthase) |
|
|
Term
|
Definition
| Glucocorticoid production |
|
|
Term
|
Definition
|
|
Term
| Adrenal medulla produces: |
|
Definition
|
|
Term
|
Definition
| Peptide hormone derived by proteolytic cleavage of POMC (proopiomelanocortin) |
|
|
Term
| Adrenal cortex production |
|
Definition
|
|
Term
|
Definition
| --> DHT deficiency; DHT is required for prenatal development of external male genitalia; hyponarremia, hyperkalemia |
|
|
Term
| 17a-Hydroxylase deficiency |
|
Definition
| Only mineralocorticoid production; hypertension (increased aldosterone) |
|
|
Term
| 21a-Hydroxylase deficiency |
|
Definition
| Most common; lack of glucocorticoids, mineralocorticoids; overproduction of androgens --> masculinization of female external genitalia; forms: 1) severe = salt-wasting --> ambiguous genitilia in female 2) simple virilizing 3) non-classic form = mildest --> production of androgens = infertility |
|
|
Term
| 11B-Hydroxylase deficiency |
|
Definition
| Virilization, masculinization; deoxycorticosterone acts as mineralocorticoid --> low renin hypertension; note: no cortisol --> adrenal hyperplasia |
|
|
Term
|
Definition
| Hypersecretion of ACTH by pituitary tumor (pituitary dependent) |
|
|
Term
|
Definition
| Associated with increased corticosteroid levels |
|
|
Term
|
Definition
| Adrenocortical failure --> decreased production of glucocorticoids, mineralocorticoids --> increased ACTH levels |
|
|
Term
| First step of lipid digestion |
|
Definition
| Initiated by lingual lipase, acid stable lipase --> targets TAG with butyric (4C) and capric (10C) acid |
|
|
Term
| Second step of lipid digestion |
|
Definition
| Emulisification with bile salts (glycocholic and taurocholic acid) --> formation of mixed micelles (increase surface area) |
|
|
Term
| Thrid step of lipid digestion |
|
Definition
| Pancreatic lipases catalyze hydrolysis of TAG (C1, C3) to form DAG, MAG |
|
|
Term
|
Definition
| Aids in binding of pancreatic lipase |
|
|
Term
|
Definition
| Inhibits gastric and pancreatic lipase --> prevents absorption of fat (steatorrhea) |
|
|
Term
| Entry of shot, medium chain lipids at intertinal mucosa |
|
Definition
| Enter directly w/o micelle assistance, not esterified |
|
|
Term
| Entry of long chain lipids at intertinal mucosa |
|
Definition
| Sequestered in cytosol via intestinal FA binding protein (I-FABP) |
|
|
Term
| ApoB-48; synthesis location: |
|
Definition
| [cholesterol transport, clearance]; RNA editing of ApoB-100; incorporated by MTP (microsomal TAG transfer protein); synthesized in RER of intestinal muscoa only; chylomicrons |
|
|
Term
|
Definition
| Mature in blood via transfer of ApoC11, ApoE from HDL; TAG = major component |
|
|
Term
|
Definition
| Activates lipoprotein lipase (bound to capillary wall via heparin domain and GPIHBPI) |
|
|
Term
|
Definition
| GPI anchored high density lipoprotein binding protein |
|
|
Term
|
Definition
Transfers FA, glycerol, chylomicron remnants to liver and endocytosed
Enhances proteolytic breakdown of B-amyloid plaques (ApoE4 varient increases Alzheimer disease risk) |
|
|
Term
|
Definition
| Interacts with chylomicrons to provide Fas to muscle and adipose |
|
|
Term
|
Definition
| RBC, nervous tissue, brain, liver |
|
|
Term
| Lipoprotein lipase (LPL) deficiency |
|
Definition
| Chylomicronemia = increased fat in blood |
|
|
Term
| Cosubstrate for esterification into TAG |
|
Definition
|
|
Term
| Tissue that lacks glycerokinase |
|
Definition
|
|
Term
| Defects in lipid absorption |
|
Definition
| Decrease in essential fatty acids --> decreast in fat soluble vitamins (A, D, E, K) |
|
|
Term
|
Definition
| TAG, cholesterol, phospholipids |
|
|
Term
| Composition of lipoproteins |
|
Definition
| Cholesterol composition: HDL > LDL > VLDL > chylomicron (inverse for TAG composition) |
|
|
Term
|
Definition
| Structural portein of chylomicrons, VLDL, LDL (NOT HDL) |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| Disposal of chylomicron remnants |
|
Definition
1) binds heparin sulfate proteoglycans
2) binds lipoprotein receptors on hepatocyte (requires ApoE)
3) complex is endocytosed in Space of Disse
4) hydrolysis via lysosomal enzymes |
|
|
Term
|
Definition
| Regulates lipoprotein lipase and lecithin-cholesterol acyl transferase; facilitates transfer of lipids between lipoprotein classes; mediates cellular uptake (binds cell surface receptors) |
|
|
Term
|
Definition
| Delivers de novo TAG to peripheral tissues (TAG released via lipoprotein lipase); precursor to LDL |
|
|
Term
|
Definition
Delivers cholesterol to peripheral tissues and liver
Highest cholesterol content
Single ApoB-100 initiates removal via receptor mediated endocytosis |
|
|
Term
|
Definition
In sinusoidal space of Disse in liver
Attached to luminal surface via heparin sulfate
Generates LDL, IDL, VLDL; generation of HDL3 via HDL |
|
|
Term
| Nascent VLDL (similarities to chylomicrons) |
|
Definition
| Acquires ApoE and ApoC from HDL |
|
|
Term
| Nascent VLDL (dis-smiliarities to chylomicrons) |
|
Definition
|
|
Term
| Generation of IDL from VLDL |
|
Definition
| VLDL --> IDL (lipoprotein lipase); IDL --> LDL (hepatic TAG lipase) |
|
|
Term
|
Definition
| On macrophages, endothelial cells; lower affinity than LDL-R (greatest contribution at increased plasma LDL levels); acetylation/oxidation of LDL increases affinity of scavenger receptors |
|
|
Term
|
Definition
| Myeloperoxidase produced in leukocytes |
|
|
Term
Increased cholesterol levels:
1) Stimulate
2) Reduce |
|
Definition
Stimulates ACAT (acyl-CoA cholesterol acyl transferase)
Reduces cholesterol synthesis, LDL-R synthesis |
|
|
Term
| Free, unesterified cholesterol acts on: |
|
Definition
Induces ACAT (converts free cholesterol into insoluble cholesterol esters)
Represses HMG-CoA reductase (rate limiting step of cholesterol biosynthesis)
Reduces LDL-R synthesis/ accelerates degradation
Mild down-regulation of scavenger receptors at increased plasma cholesterol levels (macrophages accumulate cholesterol) |
|
|
Term
| Reverse transport of cholesterol |
|
Definition
| HDL transports cholesterol from extrahepatic tissues to liver |
|
|
Term
|
Definition
| Released from liver, intestine; contains ApoA1, ApoA2, ApoC; (intestinal HDL formed with only ApoA1) |
|
|
Term
|
Definition
| Component of chylomicrons, VLDL, LDL only |
|
|
Term
| Lecithin-cholesterol acyl transferase |
|
Definition
| Insoluble; binds HDL; activated by ApoA1 |
|
|
Term
| Cholesterol ester transfer protein (CETP) |
|
Definition
| Transfers cholesterol esters from HDL to other lipoproteins; stimulated by increase in fatty acids |
|
|
Term
|
Definition
| Essential for endocytosis |
|
|
Term
|
Definition
| LDL disulfide bonded to ApoB-100 (elevated Lpa = independent risk factor for atherosclerosis) |
|
|
Term
|
Definition
| AR; deficiency in MTP (microsomal TAG transfer protein) --> decrease absorption of dietary fats, cholesterol, fat soluble vitamins (A, D, E, K) --> ApoB containing lipoproteins (VLDL, LDL, chylomicrons) absent |
|
|
Term
|
Definition
| Binds ApoA1 on cell surface for transfer of free cholesterol (ApoA1 transferred to nascent HDL) |
|
|
Term
|
Definition
| AR; orange tonsile; mutation in ABCA1 (ATP binding cassette gene) --> deficiency of ApoA1, ApoA2 of HDL |
|
|
Term
| Famialial Hypercholesterolemia |
|
Definition
| AD; xanthomas; LDL-R mutation --> elevated total cholesterol, LDL |
|
|
Term
| Famialial Hypercholesterolemia Treatment: Cholestyramine |
|
Definition
| Decreases serum cholesterol w/ bile acid absorbing resins (increases cholesterol-7-a-hydroxylase) |
|
|
Term
| Famialial Hypercholesterolemia Treatment: Lovastatin |
|
Definition
| Decreases HMG-CoA reductase --> lowers endogenous cholesterol |
|
|
Term
| Famialial Hypercholesterolemia Treatment: Ezetimibe |
|
Definition
| Decreases cholesterol absorptrion |
|
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Term
|
Definition
Decreases VLDL, LDL, TAG lipase
Increases HDL, lipoprotein lipase |
|
|
Term
|
Definition
| Binds peroxisome proliferator activated receptors (PPAra) |
|
|
Term
|
Definition
| Increases lipoprotein lipase, HDL; decreases TAG lipolysis, FFAuptake, VLDL/TAG synthesis |
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Term
|
Definition
| AR; cholesterol acquired by HDL cannot be esterified --> elevated free cholesterol:cholesterol ester in lipoproteins |
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Term
|
Definition
| Increase in HDL cholesterol |
|
|
Term
| Hyperlipoproteinemia Type I: Hyperchylomicronemia |
|
Definition
| Impaired TAG hydrolysis, deficiency to lipoprotein lipase, ApoC-11 |
|
|
Term
| Hyperlipoproteinemia Type II: Hypercholesterolemia |
|
Definition
| Elevated VLDL, LDL (LDL-R mutation) |
|
|
Term
| Hyperlipoproteinemia Type III: Dysbetalipoproteinemia |
|
Definition
| Increase chylomicron, VLDL remnants; ApoE cannot bind mutated ApoE-R |
|
|
Term
| Hyperlipoproteinemia Type IV: Hyperlipoproteinemia |
|
Definition
|
|
Term
| Hyperlipoproteinemia Type V: Hyperlipoproteinemia |
|
Definition
| Increase in VLDL, chylomicrons (mutated ApoA5) |
|
|
Term
| Strongest reducing agent (loss of e-) |
|
Definition
|
|
Term
| Strongest oxidizing agent (gain of e-) |
|
Definition
|
|
Term
|
Definition
| Attached to S of proteins |
|
|
Term
|
Definition
| attached by hydrophobic interactions to membrane lipids |
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