Biochem Final

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Niacin: From Tryptophan via IDO (cancer drug target), defect results in Pellagra
Creatine Kinase (CK-MB): Early detector for heart attack
PE to PS: PE ---> PS (ethanolamine replaced by serine)
Cholesterol Ester Transfer Protein (CETP): HDLs, Transfer CE in HDLs to LDLs, IDLs, and VLDLs
Refeeding Syndrome: Lack of digestive enzymes, intracellular K+ depleted (needed for ATP formation)
Glutamate: From Glutamine via glutaminase OR AA + alpha-KG via transamination
Pyruvate Carboxylase: Pyruvate + CO2 + ATP ---> OAA + ADP (in mitochondria) (gluconeogenesis)
Niacin: B3, NAD+, NADP+
Carnitine deficiency: in muslces, accumulation of TAGs in mucles
Heme degradation: RBC, Heme --> biliverdin (via heme oxygenase) --> bilirubin (NADPH + biliverdin reductase)
Maltose: Broken down by maltase into 2 glucose
Viagra: inhibits cGMP, maintains muscle relaxation and opened blood vessels
Sphingolipids: Membrane lipids, long-chain sphingosine, NO glycerol, precursor = ceramide
Extracellular Fluid: 20% (15% Interstitial, 5% plasma), Anions - Cl-, HCO3+, Cations - Na+
Apo E: LDL Receptor, Chylomicron remnant receptor, Transferred between lipoproteins
Cholesterol: 27 C, membran component, biosynthetic precursor, Cholesteryl esters - storage and transport
Glycogen break down: Glucagon increases cAMP, activates PKA, activates phosphorylase kinase, phosphorylates glycogen phosphorylase, glycogen degradation
Fructosuria: Lack of fructokinase (Benign)
Glutathione: Glu-Cys-Gly, intracellular reducing agent, in liver, Oxidized = GSSG in RBC
Osteogenesis imperfecta: Brittle bone disease, 4 types
FA Biosynthesis: In cytosol, Condensation and elongation
Glucogenic AA: Form pyruvate, alpha-KG, succinyl-CoA, fumarate, OAA (can be used in gluconeogenesis), Phenylalanine, Tyrosine, Tryptophan, Isoleucine, Threonine
Activation of Sugars: Sugar binds to nucleotide, NDP sugar transferred to growing chain
Proline: Causes problems for alpha helices
Scurvy: Lack of vitamin C (ascorbic acid), defects in collagen
Glycogen breakdown regulation: Glucagon/epi bind to receptors on cell surface, activate adenylate cyclase, increases cAMP, increases PKA, PKA activates phosphoylase kinase (phosphorylates glycogen phosphate), degradation of glycogen
Phosphlipases: Hydrolyze phospholipids, release arachidonic acid - precursor of prostanoids, cleavage of PIP2
FA Synthase complex: 7 enzymes, 2 binding sites (B site - pantothenic acid Vit 5 & C site - cystein residue)
Catecholamines: Signaling molecules (Dopamine, Norepinephrine, Epinephrine) from Tyrosine (need THBtn and SAM)
Increased Na: Dehydration
Hyperammonemia: Defect in carbamoyl phosphate synthetase I or ornithine transcarbamoylase
Megaloblastic Anemia: Prevents DNA replication, folate deficiency also, B12 deficiency, N5-methyl THF accumulates
Acute Intermittent Porphyria (AIP): Uroporphyrinogen Synthase deficiency, ALA and PBG accumulate, Dark red urine, Confusion and sharp abdominal pain
Lesch-Nyhan Syndrome: Overproduction of uric acid, purines accumulate and damage CNS, HGRPT absent (salvages hypoxanthine and guanine)
Lipoproteins: Delivery of lipoproteins to tissu/organs, outer monolayer shell of phospholipids, cholesterol, inside core of TAGs and cholesteryl esters
Glucose Phosphate Isomerase: Glucose 6-phosphate ---> Fructose 6-phosphate (glycolysis)
Hartnup Disease: Blocks activity of AA transporter of large neutral AAs, Kidney - reabsorption of neutral AA from ultra filtration, malabsorption of dietary neural AAs (go into urine), Resembles Pellagra, supplement with Niacin
Uncouplers of oxidative phosphate: Inhibits ATP formation but allows e- and H pumping, energy lost as heat, DNP, Brown fat, Cyanide
CAC: generates 3 NADH, 1 FADH2, 12 ATPS, Mit, major ATP-gen pathway
Gaucher's: Cerebroside build up (sphingolipidoses)
Whooping Cough: Inhibits Gi, cAMP rises
niacin: Inhibits VLDL synthesis in liver, decreases LDLs
Irreversible Inhibitors: Chemically modifies permanently, doesnt change Km, decreases Vmax, ex - Aspirin
Nieman-Pick: Sphingomyelin build up (sphingolipidoses)
Purine degradation: 5' nucleotidase and purine nucleoside phosphorylase, for adenosine - need AMP deaminase and adenosine daminase
Cholesterol biosynthesis steps: acetyl-coA, HMG-CoA, mevalonate, isoprene units, farnesyl, squalene, cholesterol
Serine: From 3-PG ---> 3-Phosphohydroxypyruvate --> 3-phosphoserine ---> Serine
Steroid Hormones: 21 or less C, shortened side chains, additional Oh or carbonyl and double bonds
Beta-linked polysaccharaides: Structural support, glycosaminoglycans, CANNOT digest
Decreased Ca: hormonal, neuronal/muscular function
Acetyl-CoA Carboxylase: Acetyl-CoA ---> Malyonyl-CoA (Committed step) (FA biosynthesis)
Endopeptidase: Inactive zymogen pepsinogen ----> Pepsin (from chief cells)
Hepatocellular Jaundice: Liver disease, acetaminophen poisoning, no glucoronated bilirubin - increase in indirect bilirubin, pale feces and urine (increase in ALT and AST)
Ethanol (O2): Generates H2O2 and hydroxyethyl radical, DNA damage
Tyrosine: From phenyalanine via phenylalanine hydroxylase (in liver, irreversible, uses O2, NADH, and THBtn)
Liver: FA, glucose, AA, lactate. Stores glycogen
Riboflavin: B2, FAD, FMN
Phospholipase C: activated by Gq, hydrolyzes PIP2, generates IP3 and DAG
Polyunsaturated FA: conjugated double bond, inhibit hydratase, little less ATP formed because less FADs ---> FADH2
Atractyloside: Inhibits adenine nucleotide translocase, - exchanges ATP for ADP
Parkinsonism: Decrease of dopaminergic neurons (from tyrosine)
Urea Cycle: Exclusively by liver, Mit and Cyt, Carbamoyl phosphate (via Carbamoyl phosphate synthetase 1) + Ornithine ---> Citrulline (ornithine transcarbamoylase) + Aspartate ----> Arginosuccinate (via arginosuccinate synthetase) ----> Arginine + Fumarate (via arginosuccinate lyase) ---> Urea + Ornithine (arginase)
Celiac Disease: Inappropriate immune response to alpha-gliadin (from gluten), decrease in absorptive area of SI, nutrient deficiency/cramps/bloating
Pyrimidine Synthesis: Carbamoyl phosphate synthetase, aspartate transcarb., Glutamine --> Carbamoyl phosphate --> carbamoyl aspartate --> dihydroorotate --> orotate --> orotidine monophos (+ PRPP) --> UMP
SCID: Immune cells cannot proliferate, increased dATP - shuts down ribonucleotide reduction and dividing cells die
Apo B-48: Chylomicrons
Globulins: Increased in immune response
ATP Synthase Complex: Complex 5 - FoFi ATP Synthase, couples re-entry of H to ATP synthesis, Fo (proton channel) F1 (catalytic moiety)
Glycine: From Serine + THF via serine hydroxymethyl transferase (Makes N5N10 THF)
IP3: Binds receptors on Ca compartment, Ca flows into cytosol and activates PKC
Glutamate Dehydrogenase: in Liver, AA degradation (mainly striated muscle protein) Glutamate ---> alpha-KG (for TCA) + NADH (for ETS) + NH4+
Branched-Chain AA catabolism: BCAT (in muscles) and BCKDH (liver) break Valine, Leucine, and Isoleucine into Succinyl-CoA, Acetyl-CoA, and Succinyl-CoA
PEP Carboxykinase: OAA + GTP <---> PEP + GDP + CO2 (Mit and Cyt) (gluconeogenesis)
Adipose: Triglycerides (storage from glucose), Gives glycerol and FA
ILDs: Deliver TAG FA to extra-hepatic tissues, from VLDLs
Increased Ca: hormonal Or skeletal degradation
CAT deficiency: Muscle pain after exercise
Sphingolipidoses: Lysosome - site of degradation or storage disease (accumulation of glycolipid)
Guanylate Cyclase: Converts GTP to cGMP, activates cGMP-dependent protein kinase, atrial natriuretic peptides (bind guan receptors and relax smooth muscle - lowers BP)
Hypotonia: Defects in creatine synthesis, little skeletal/cardiac muscle pathology, neurological symptoms, Treat with dietary carnitine
Cholesterol Biosynthesis: Cytosol and ER membrane surface, Acetyl-CoA - precursor
Phosphoglycerate Kinase: 1,3-Bisphophoglycerate + ATP ---> 3-phophoglycerate (glycolysis)
Albinism: Decreased tyrosinase function in making melanin
Nitric Oxide (NO): Free radical, vasodilator, activation of guanylate cyclase
Fructose 1,6-bisphosphatase: PEP ---> Fructose 6-phosphate (Irreversible step) (gluconeogenesis)
Lead Poisoning: ALA synthase and Ferrochelatase inhibitor
Ehlers-Danlos: Mutations in collagen, recessive and dominant inheritance
Fumarase: Fumarate + H2O <----> Malate (CAC)
Isocitrate Dehydrogenase: Isocitrate + NAD+ -----> alpha-KG + NADH + CO2 + H (CAC - Regulation step)
DNP derivatives: Causes hyperthermia, increases heat production
NO: From Arginine, activates guanylate cyclase (converts GTP to cGMP), cGMP activates cGMP-dependent protein kinase, muscle relaxation, cGMP degraded by phosphodiesterases
Glucose 6-phosphatase: Fructose 6-phosphate ---> glucose 6 phosphate ---> glucose (LIVER) (gluconeogenesis)
Glucagon: fasting/exercise, secreted by alpha cells of the pancreas, increases blood glucose by stimulating glycogen break down
Urea (BUN): increased - loss of kidney function
Amidophosphoribosyl transferase: Committed step of de novo synthesis of purines, add amino group to 5'-phosphoribose
Glutathione: GSSG reduced to GSH (with NADPH)
Pernicious Anemia: Lack of intrinsic factor, accumulation of methylmalonyl-CoA (competitive inhibition of FA synthesis), myelin sheath synthesis decreased
McArdle Disease: Defect in glycogen phosphorylase, muscles can't used stored glycogen (glycogen storage disease)
Essential AA: Pvt Tim Hall (Phenylalanine, Valine, Threonine, Tryptophan, Isoleucine, Methionine, Histidine, Arginine, Leucine, Lysine)
Sickle Cell Anemia: Valine substitutes glutamate, hydrophobic regions, polymerizes into fibers
Gluconeogenesis: Liver and kidney, glucose from pyruvate, precursors: lactate, AA, glycerol
Alpha-linked polysaccharides: Storage form of glucose, Glycogen, CAN digest
G proteins: active with GTP, inactive with GDP, generates intracellular 2nd messengers
Peroxynitrite (ONOO-): From superoxide and peroxynitrate anions, Very reactive, covalently modifies lipids, DNA, and proteins
Intracellular Fluid: 40%, Anions - proteins, phosphate, Cations - K+
Glycolysis: Glucose ---> 2 pyruvate + 2 ATP + NADH
Synthesis of GAGs: Golgi apparatus, RER (protein moiety), Sulfates added by sulfotransferases, secreted in EC space and linked to acidic hyaluronic acid, non-covalent linkage by linker protein
Biosynthesis of TAG: glycerol-3-phosphate - Glycerol kinase (phosph. of glycerol) or Reduction of DHAP (glycerol-3-P-dehydrogenase)
Tay-Sachs: Ganglioside build up (sphingolipidoses)
Muscles: Fatty acids, glucose, ketone bodies, anaerobic/aerobic, proteins degraded into glucose
Hemochromatosis: HFE gene mutated - Iron storage disease. Ox stress, destruction of liver, pancreas, heart muscle
Oxygen: singlet O2 --> ground state O2 --> superoxide anion radical O2- --> hydrogen peroxide H2O2 --> hydroxyl radical HO + hydroxide anion (most reactive) --> water
Hyperuricemia: increase in uric acid, mostly due to undersecretion (renal insufficiency causing metabolic acidosis)
Glycogenin: Transfers glucose from UDP-glucose to nonreducing ends of chain, alpha (1,4)-glycosidic bonds, autoglycosylation (glycogen synthesis)
OAA: From pyruvate and aspartate (via aspartate aminotransferase AST)
Direct/Indirect Billirubin: Indirect - albumin bound, direct - conjugated to uronic acids....Increased - liver failure
Melatonin: From serotonin (tryptophan) via N-acetyltransferase and SAM), Light/dark cycle - pineal gland, induces sleepiness
Condensation and Elongation: Acetyl transacylation (acetyl to C site), Malonyl transacylation (3-C malonyl to B site), Condensation (liberate CO2 and C site free), Reduction (NADPH from PPP to NADP+), Dehydration (remove H2O), Reduction (to NADP+), Cleavage (Thioesterase, end product - palmitate (C-16)) (FA Biosynthesis)
Eicosanoids: Derivatives of C-20 polyunsaturated FA, Postanoids and leukotrienes (local hormones, not in RBC), release from phspholipids by PLA2
2,3-BPG: Increases interactions within Hb, favors the T-state
Asparagine: From Aspartate via Asparagine synthetase (with ATP and glutamine)
Apo B-100: VLDLs, IDLs, LDLs
Chylomicrons: Deliver lipids to tissues, to intestinal enterocytes, hydrolyzed by lipoprotein lipases in capillary walls
Aconitase: Citrate <---> Isocitrate (CAC)
Alcohol: Reduces amount of NAD in cytosol, cannot carry out gluconeogenesis because lactate cannot be ox into pyruvate. lactate accumulates and leads to metabolic acidosis
Melanin: From Tyrosine (via tyrosinase with copper) - in golgi of melanocytes
HbF: Reduced affinity for 2,3-BPG, increased oxygen affinity, serine in place of histidine
Malate Dehydrogenase: Malate + NAD+ <----> OAA + HADH + H+ (CAC)
Phosphoglyceromutase: 3-Phosphoglycerate ----> 2-Phosphoglycerate (glycolysis)
Increased phosphate: Bone disease, renal failure
PDH Complex: pyruvate decarboxylase, dihydrolipoyl transacetylase, dihydrolipoyl dehydrogenase, Pyruvate into Acetyl-CoA (uses NAD+), irreversible, Mit matrix
Cholera: Locks alpha in GTP bound state, cAMP levels rise, PKA active, phosphorylates CFTR - Cl-, Na+, H2O secreted into intestinal lumen, massive diarrhea
Catalase: 2H2O2 ----> 2 H2O + O2
Zellweger Syndrome: Poorly functioning peroxisomes, defective myelination
TAGs: Lipids stored as fatty acids of these, mainly in adipose tissue
Iron and Copper (O2): superoxide anion and hydroxyl radical produced
Branching Enzymes: Fragments from alpha (1,4) glucose chain to C6 of another chain, glycogen synthase - branch elongation (glycogen synthesis)
DNA ROS damage: Hydroxyl radical --> strand breaks, interferes with replication and transcription
Glycogen Synthesis: Liver and muscle cells (for body and self, respectively), cytosol
Pyruvate Kinase: Phosphoenolpyruvate ---> Pyruvate + ATP (glycolysis)
Non-competitive Inhibitors: Reversible, binds at sites other than active site, doesnt change Km, decreases Vmax
Gout - Gold Standard: negatively birefringement needle-shaped crystals under polarized microscopy. Yellow - parallel, blue - perpendicular, want to achieve 6.4 level
Activation of FA: Very long chains (peroxisomes), long chains (cytosol), Med-short chains (mitochondria)
Phosphofructokinase I: Fructose 6-phosphate + ATP ---> Fructose 1,6-bisphosphate (glycolysis)
Lecithin-Cholesterol acyl transferase (LCAT): Plasma - bound to HDLs, FA from PC ---> CE in cholesterol
Epinephrine: Quick stress, secreted from adrenal medulla, increases blood glucose levels ---> ATP production, muscles respond strongly
Unsaturated FA: Isomerase converts cis double bond to trans
Alanine: From pyruvate + glutamate via Alanine Aminotransferase (ALT) (also makes Alpha-KG)
Phenylketonuria (PKU): Unable to convert phenylalanine to tyrosine, defects with phenylalanine hydroxylase and dihydrobiopterin reductase, Mental retardation/seizures/hypopigmentation/eczematous skin rashes, Inhibits transport across blood/brain barrier of other AAs which decreases synthesis and increased degradation of myelin
Maple Syrup Urine Disease: Accumulation of branched-chain alpha-ketoacids, Defects in BCKDH
RBC: Glycolysis and Fermentation, anaerobic
RBC: Anaeorbic, glucose --> lactate
AST: Liver, heart, skeletal muscle, kidney, brain...
Aspartate: From OAA + Glutamate via Aspartate Aminotransferase (AST) (also makes Alpha-KG)
Competitive Inhibitors: Reversible, Most common, competes with substrate, increases Km, doesnt change Vmax, ex - Advil and Tylenol
Hexokinase: Phosphorylates C-6 of glucose - destabilizes it and initiates breakdown
Serum albumin: Needed to be bound to FA to be transported in circulation, non-covalent bond, "free FA"
Brain: No stores, glucose, ketone bodies - starvation
3 categories of glycolipids: Cerebrosides (1 sugar, no charge), Globosides (2-4 sugars, no charge), Gangliosides (several sugars, sialic acid (negative charge))
Cyanide: ET inhibition (complex 4) Prevents Fe3+ reduction, lactic acidosis - death
Allopurinol: xanthine oxidase inhibitor for overproduction of uric acid
Acetyl from mit to cyto: Condense acetyl-CoA with OAA to citrate, citrate transported via translocase, cleaved by citrate lyase
CAT II: Inner membrane, exchanges carnitine of fatty acyl-carnitine with CoA to reform FA acyl-CoA inside mit matrix
Sphingomyelin: PC + Ceramide ---> DAG + Sphingomyelin
Decreased K: vomiting, diarrhea
TMP: unique to DNA, meth of dUMP --> dTMP (Thymidylate synthase) needs THF
Apo A-1: HDLs, activator of LCAT
Porphyria Cutanea Tarda (PCT): Most Common, Uroporphyrinogen Decarboxylase deficiency, buildup of porphyrins, Skin photosensitivity - blistering
Apo C-II: Lipoprotein lipase activator, chylomicrons, VLDL, LDL, HDLs - plasma reservoir
Familial Hypercholesterolemia (FH): Increases LDLs, deficiency of LDL receptors
Lipoprotein Lipase (LPL): In capillary walls, hydrolyzes TAGs
Xanthine oxidase: Free bases --> Xanthine ---> uric acid (purine degradation)
PPP: Provides NADPH and Ribose for biosynthetic reactions, degeneration of glucose
Post-hepatic Jaundice: Cholestatic (obstruction), Obstruct biliary duct, pale feces and intense urine, Increase in direct bilirubin
AA Pool: Sum of extracellular and intracellular AAs within body, for energy metabolism or protein synthesis
Prehepatic Jaundice: Hemolytic, increased RBC destruction --> increased serum bilirubin, exceeds liver's glucuronation and excretion, Increase in indirect bilirubin
FA Transport: FA from CoA to carnitine via transferase and taken into mit by fatty acyl-carnitine translocator, for C12 and up
B12: From bacteria (meats and shellfish), Released in presence of gastric acid and pancreatic proteases, binds to intrinsic factor
Bile Acid Sequesterants: Interrupt reabsorption of bile acids, more made, increases cholesterol elimination
Creatinine: Dependent on muscle mass, kidney function, increased - glomerular filtration problem
Glyceraldehyde phosphate dehydrogenase: GAP + NAD ----> 1,3-Bisphophoglycerate + NADH (glycolysis)
Purine metabolism: PRPP synthase and amidophosphoribosyl transferase, needs THF
PRPP Synthase: PPP ---> PRPP in purine metabolism
Fructose 2, 6-bisphosphate: Stimulates PFK1 which stimulates glycolysis, stimulated by fructose 6-phosphate (with AMP)
Ribonucleotide Reductase: transfers e- from thioredoxin to the C2 carbon of ribose of nucleotide diphosphate (e- from NADPH)
Vioxx: Inhibits prostaglandin synthase only (COX1 and COX2)
Alpha-KG Dehydrogenase Complex: A-KG + CoASH + NAD+ ---> Succinyl-CoA + CO2 + NADH + H+ (CAC - Regulation step)
Protein ROS damage: Causes formation of incorrect disulfide bonds
Sodium: extracellular, reflects oncotic pressure
Committed step in Cholesterol biosynthesis: HMG-CoA Reductase, HMG-CoA ---> Mevalonate (committed step)
VLDLs: deliver TAG FA to extra-hepatic tissues
GAGs: Repeating units of aminosugar and an acid, Negative charge (entrapment of water), in ECM
Enolase: 2-phosphoglycerate ---> Phosphoenolpyruvate (glycolysis)
PA Pathway: Formation of PI
LDLs: Deliver cholesterol and CE from circulation to tissues/organs (to liver too), deliver via endocytosis by LDL receptor
Glutamine: From Glutamate via glutamine synthetase (ATP and ammonium ions (removal))
Troponins: Most sensitive marker for MI
Glycerol Phosphate Shuttle: Muscle and Brain tissue, e- to FAD ---> FADH, 2 molecules of ATP, less effective
Serotonin: From Tryptophan, signaling molecule, via tryptophan hydroxylase and THBtn
Van Gierke Disease: Defect in glucose 6-phosphatase, incapable of exporting glucose, hypoglycemia and increases glycogen in liver (glycogen storage disease)
Statins: Inhibit HMG-CoA Reductase (reduces cholesterol biosynthesis), increases LDL Receptors (increases LDL clearance)
Bile Acids: Formed in liver, stored in gallbladder, utilize NADPH, cholate, chenodeoxycholate (additional OH), shortened side chain, saturated rings (no double bonds)
CO: Reacts with Hb, prevents O2 loading in lungs
Decreased Na: water retention
Xanthine Oxidase (O2): 2 H2O2 produced
Chemiosmotic Model: Active transport by ETS of H out matrix, electrochemical gradient, H flow down gradient, free energy "trapped" to form ATP
Bubonic Plague: Yoph - protein tyrosine phosphatase, desphosphorylates key proteins in immune response
Citrate Synthase: Acetyl-CoA + OAA ---> Citrate + CoASH (CAC)
Atherosclerosis: Increased homocysteine
Myasthenia Gravis: Autoimmune disease, antibodies against nicotinic acetylcholine receptor
Gout: Precipitation of uric acid crystals
Albumin: Binds hydrophobic molecules and Ca++, maintains oncotic pressure, lowered - edema (liver disease)
Succinyl-CoA Synthetase: Succinyl-CoA + GDP + Pi <---> Succinate + GTP (CAC)
HDLs: Reverse transport of cholesterol, formed in liver, uptake of free cholesterol, esterification of Chol (by LCAT), transfer of CE to plasma lipoproteins to LDLs (by CETP)
Increased K: Renal insufficiency
DAG: activates isoforms of PKC
Carnitine Translocase (CT): Transfers acyl-carnitine into mit matrix in exchange for free carnitine
Uric Acid: end product of purine degradation, increased - excretion problems --> gout
Heart: Constant energy and feeding, aerobic only, fatty acids
G6PD: limits PPP, decreases glutathione which protects RBC against oxidative stress
Fructose Bisphosphate Aldose: Fructose 1,6-bisphosphate ---> DHAP and GAP (glycolysis)
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: Most common, disorder of liver beta oxidation, avoid fasting
PFK2: Inhibited by cAMP, fasting
Decreased phosphate: impairs glucose metabolism - refeeding syndrome
Beta oxidation of FA: Mit matrix, Dehydrogenation (Acyl-CoA dehydrogenase, FADH2), hydration (hydratase), Dehydration (NADH), Cleavage (Thiolase, liberates acetyl-CoA)
Increased Alkaline phosphatase: bone or liver disease, with increase in bili - liver, decrease in bili - bone
THF: Acceptor for 1-C groups, most ox (N10-formyl THF), msot reduced (N5-methyl THF)
Glycogen Degradation: Glycogen phosphorylase activated, alpha (1,4)-glycosidic bond in glycogen replaced by phosphate bond, phosphoglucomutase, debranching enzyme
Cysteine: from Methionine --> SAM --> S-adenosylhomocystein ---> Cysteine (needs ATP)
Ketogenic AA: Form acetyl-CoA or acetoacetyl-CoA (form ketone bodies), Leucine and Lysine
Thyroid hormones: From Tyrosine - iodinated, T3 and T4 via throid peroxidase
Cyclooxygenase Pathway (COX): Prostaglandin synthase, cyclooxygenase, hydroperoxidase, formation of PGH, COX-1 (constitutive enzyme), COX-2 (inducible enzyme - inflammation)
Lactose: Broken down by lactase into glucose and galactose
Malate-aspartate Shuttle: Liver and Heart, NADH, 3 molecules of ATP, more effective
DAG Pathways: Formation of PE and PC (PE ---> PC uses SAM)
Nicotinic Acetylcholine Receptor: Ligand-gated Na+ channel, 2 alpha, 1 beta, 1 gamma, 1 delta, acetylcholine binds receptor (alpha), conformational change occurs, channel opens and Na+ moves down concentration gradient into cell
Glutathione Peroxidase: H2O2 + 2GSH ---> 2H2O + GSSG
Proteoglycans: Core protein, attached carbohydrate side chains (GAGs), in ECM
Hyperglycemia: Generates Sorbitol (cataracts) and depletes NADPH (decreases antioxidation defense)
Mucopolysaccharidosis (MPS): Proteoglycan-degrading hydrolases defective, accumulate in lysosomes, Hunter's, Hurler-Scheie, Sanfilippo's
Chylomicrons: Released into lymph and blood, FAs released by lipoprotein lipase in capillaries, get smaller into remnants taken up by liver via receptor-mediated endocytosis
Ezetimibe selectivity: Inhibits intestine absorption of cholesterol in SI
Fructose Metabolism: In liver, Fructose 1-phosphate by fructokinase. To DHAP in glycolysis
Cori Disease: Defect in glycogen debranching enzyme, glycogen granules become large (Glycogen storage disease)
Activation of glucose: Hexokinase, Phosphoglucomutase, UDP-glucose pyrophosphorylase ---- makes UDP-glucose from glucose (glycogen synthesis)
Enzyme Complexes: 1 (NADH-CoQ) 2 (Succinate-CoQ) 3 (CoQH2 - Cyt c) 4 (Cytochrome oxidase), 1, 3, 4 coupled to ATP formation
Membrane ROS damage: Peroxidation of PUFAs. lipid peroxides unstable and abstract e- from PUFA double bond
Galactosemia: Lack of galactokinase (mental retardation)
Superoxide Dismutase: 2O2- + 2H+ ----> H2O2 + O2
Membrane glycerophospholipids: PI (phophatidylinositols - tail), PE (phosphatidylethanolamine - carries head), PC (phosphatidylcholine - carries head), PS (phosphatidylserine)
Pressure Ulcers: Treated with Arginine
Sucrose: Broken down by sucrase into glucose and fructose
Cori Cycle: Lactate transported to liver, lactic acid ---> pyruvate, pyruvate ---> glucose, glucose in blood for glycolysis
Vitamin D: UV Photolysis, active calcitriol by hydroxylation in liver and kidney
Atherosclerosis: buildup of deposits in blood vessels, increased LDL:HDL, dietary restriction
Insulin: Secreted by beta cells of pancrease, decreases blood glucose by glycogen deposits in liver and muscle
Salvage: Purines from diet and recyled - free bases bond to PRPP, Cytosines NOT salvaged
Hemolytic anemia: Favism, H202-producing drugs, PPP deficiency
Carnitine-acyl transferase 1 (CAT1): Outer membrane, exchanges CoA of FA acyl-CoA with carnitine to form fatty acyl-carnitine
Acute Pancreatitis: Inflammatory disease, enzymes activated IN pancreas, alcohol/infections/gallstones, treat with fasting
Heme synthesis: Succinyl-CoA + glycine --> aminoevulinic acid (ALA synthase) ALA --> Cytosol & joins other ALA to form PBG (porphobilinogen synthase) 4 PBGs join ---> Uroporphyrinogen (uroporphyrinogen synthase) Modified and Fe2+ inserted by ferrochetelase
Propionyl-CoA: Isoleucine, Valine, Threonine, Methionin (glucogenic), Requires B12, makes Succinyl-CoA
Fructose Intolerance: Lack of aldolase (Benign)
Allopurinol: inhibits production of uric acid, inhibits xanthine oxidase, yields hypoxanthine and xanthine
ALT: Liver - increases with liver disease
Ketone Bodies: Can cross blood/brain barrier, "glucose sparing"
Succinate Dehydrogenase: Succinate + FAD <---> Fumarate + FADH2 (CAC)
Guanine deaminase: Guanine --> Xanthine (for uric acid metabolism)
Thiamin Deficiency: Low PDH and A-KG dehydrogenase, elderly, low income, alcoholics, mental confusion, ataxia, loss of eye coordination, Wernicke-Korsakoff Syndrome, Beri-Beri
Alpha-KG: From glutamine ---> glutamate ---> alpha-KG

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