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