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| 10496538099 | Glycine | Non-polar amino acid *Smallest side chain* Heme synthesis + Succinyl CoA Purine and creatinine synthesis Conjugated to *bile acids, drugs, and other metabolites* *Major inhibitory* neurotransmitter in *spinal cord* | 0 | |
| 10496538100 | Alanine | Non-polar amino acid Carrier of *ammonia* and *carbons of pyruvate from skeletal muscle to liver* | 1 | |
| 10496538101 | Valine Leucine Isoleucine | Non-polar amino acid Branched-chain amino acid whose metabolites accumulate in MSUD | 2 | |
| 10496538102 | Phenylalanine | Non-polar amino acid Accumulates in phenylketonuria Catalyzed to tyrosine via *phenylalanine hydroxylase* | 3 | |
| 10496538103 | Tryptophan | Non-polar amino acid *Largest side chain* Precursor for *niacin, serotonin, melatonin* | 4 | |
| 10496538104 | Methionine | Non-polar amino acid Involves in transfer of *methyl groups* Precursor of homocysteine | 5 | |
| 10496538105 | Proline | Non-polar amino acid Imino acid Fibrous structure of collagen and interrupts a-helices in globular proteins | 6 | |
| 10496538106 | Serine Threonine Tyrosine | Uncharged polar amino acid Hydroxyl group O-linked glycosylation and phosphorylation of proteins | 7 | |
| 10496538107 | Asparagine | Uncharged polar amino acid Carbonyl and amide group for H-bonding Site for *N-linked glycosylation of proteins* | 8 | |
| 10496538108 | Glutamine | Uncharged polar amino acid Carbonyl and amide group for H-bonding Deaminated by *glutaminase* resulting in formation of ammonia Major carrier of nitrogen to the liver from peripheral tissue | 9 | |
| 10496538109 | Cysteine | Uncharged polar amino acid Contains sulfhydryl group that is an active part of many enzymes Biosynthesis of CoA 2 form a *cystine* | 10 | |
| 10496538110 | Aspartate | Acidic amino acid Negatively charged at neutral pH Participates in ionic interaction Serves as proton donors | 11 | |
| 10496538111 | Glutamate | Acidic amino acid Negatively charged at neutral pH Participates in ionic interaction Serves as proton donors *Precursor for GABA and glutathione* | 12 | |
| 10496538112 | Arginine | Basic amino acid Positively charged at neutral pH *Precursor fo creatinine, urea, and NO* | 13 | |
| 10496538113 | Lysine | Basic amino acid Positively charged at neutral pH | 14 | |
| 10496538114 | Histidine | Basic amino acid No charge at neutral pH Used in the diagnosis of *folic acid* deficiency Concentration is dependent on circadian rhythm | 15 | |
| 10496538115 | Cytochrome C | Important for O2 transfer in the ETC | 16 | |
| 10496538116 | Uniquinone (CoQ) | The only non-protein component of the ETC | 17 | |
| 10496538117 | ↑ NADH/NAD+ and FADH2/FAD ratios ↓ oxygen consumption ↓ ATP | Effects of ETC inhibitors | 18 | |
| 10496538118 | Uncouplers | Increases permeability of the inner mitochondrial membrane to protons ↑ NADH/NAD+ and FADH2/FAD ratios ↓ oxygen consumption ↓ ATP Ex. aspirin | 19 | |
| 10496538119 | Barbiturates Piericidin A Amytal Rotenone | Complex I Inhibitors | 20 | |
| 10496538120 | Malonate Carboxin TTFA | Complex II Inhibitors | 21 | |
| 10496538121 | Antimycin A Dimercaprol | Complex III Inhibitors | 22 | |
| 10496538122 | Cyanide CO Sodium Azide Hydrogen Sulfide | Complex IV Inhibitors | 23 | |
| 10496538123 | ATPase Synthase Inhibitors | Inhibits Complex V Proton gradient continues to rise, but no escape valve ETC eventually stops Ex. Oligomycin | 24 | |
| 10496538124 | Reactive Oxygen Species | Produced by neutrophils to kill bacteria May be increased in reperfusion injury | 25 | |
| 10496538125 | Oligomycin | *Directly inhibits ATP synthase (Complex V)* Proton gradient increases but there is no "escape valve" | 26 | |
| 10496538126 | Atractyloside | Inhibits the oxidative phosphorylation by *inhibiting the transporter of ADP into and ATP out of the mitochondrion* | 27 | |
| 10496538127 | cAMP | Second messenger Protein kinase A Ex. glucagon, epinephrine (B, a2) | 28 | |
| 10496538128 | IP3/DAG | Second messenger Protein kinase B Ex. epinephrine (a1) | 29 | |
| 10496538129 | Tyrosine Kinase | Second messenger Protein kinase C Ex. ANP, NO | 30 | |
| 10496538130 | Intracellular | Second messenger Direct entry into the cell and nucleus Ex. glucocorticoids, thyroid hormones | 31 | |
| 10496538131 | GLUT 1 | GLUT Brain, kidney, colon, placenta, RBC *Glucose uptake* | 32 | |
| 10496538132 | GLUT2 | GLUT Liver pancreas, small intestines, kidney *(basement membranes)* *Rapid uptake or release of glucose* | 33 | |
| 10496538133 | GLUT3 | GLUT *Brain (more compared to GLUT1)*, kidney, pancreas *Glucose uptake* | 34 | |
| 10496538134 | GLUT4 | GLUT Heart and skeletal muscle, adipose tissue Insulin-stimulated glucose uptake | 35 | |
| 10496538135 | GLUT5 | GLUT Small intestine *lumen* - 5 letters Absorption of *fructose* | 36 | |
| 10496538136 | SGLT-1 | SGLT Small intestine and kidney Na dependent active uptake of glucose against a concentration gradient | 37 | |
| 10496538137 | Glycolysis | Glucose metabolism that converts glucose to 3 carbon compounds to produce energy Location: occurs in the cytoplasm Substrate: glucose End products: 2 molecules of *pyruvate* or *lactate* Rate limiting enzyme: phosphofructokinase-1 | 38 | |
| 10496538138 | Phosphofructokinase-1 | Rate limiting enzyme of Glycolysis Converts Fructose-6-Phosphate to Fructose-1,6- Bisphosphate Down regulated by ATP and citrate (energy not needed) Effector: *Fructose-2,6-bisphosphate* | 39 | |
| 10496538139 | Fructose-2,6-bisphosphate | Activator of *PFK-1* | 40 | |
| 10496538140 | Hexokinase | Catalyzes the phosphorylation of glucose to form glucose-6-phosphate in the first step of glycolysis Feedback-inhibited by *glucose-6-P* *Low Km and Vmax; high affinity* | 41 | |
| 10496538141 | Glucokinase | Catalyzes the phosphorylation of glucose to form glucose-6-phosphate in the first step of glycolysis Feedback-inhibited by *fructose-6-P* *High Km and Vmax; low affinity* | 42 | |
| 10496538142 | 1.5 ATP | ATP yield of NADH via *glycerophosphate shuttle* | 43 | |
| 10496538143 | Glycerophosphate Shuttle | Shuttle which ATP yield of 1.5 ATP | 44 | |
| 10496538144 | 2.5 ATP | ATP yield of NADH via *malate aspartate shuttle* | 45 | |
| 10496538145 | Malate Aspartate Shuttle | Shuttle which ATP yield of 2.5 ATP | 46 | |
| 10496538146 | Lactate Dehydrogenase | Enzyme Converts pyruvate to *lactate* | 47 | |
| 10496538147 | Pyruvate Decarboxylase | Enzyme Fermentation Converts pyruvate to *ethanol* | 48 | |
| 10496538148 | Arsenic | Competes with inorganic phosphate as a substrate for glyceraldehyde-3-P dehydrogenase *Binds to lipoic acid* | 49 | |
| 10496538149 | Fluoroacetate | *Inhibits aconitase* leading to accumulation of *citrate* | 50 | |
| 10496538150 | Isocitrate dehydrogenase a-ketoglutarate dehydrogenase Malate dehydrogenase | Enzymes in the Citric Acid Cycle that are involved in reactions that produce *NADH* | 51 | |
| 10496538151 | Succinate dehydrogenase | Enzyme in the Citric Acid Cycle that are involved in reactions that produce *FADH2* | 52 | |
| 10496538152 | 1. Thiamine - B1 2. FAD - B2 3. NAD+ - B3 4. Coenzyme A (Pantothenic acid) - B5 5. Lipoic acid | Coenzymes of *Pyruvate Dehydrogenase* Shared with a-Ketoglutarate | 53 | |
| 10496538153 | Citrate | TCA intermediate Delivers acetyl CoA to the cytosol for fatty acid synthesis | 54 | |
| 10496538154 | Succinyl CoA | TCA intermediate Used for heme synthesis and activation of ketone bodies in extrahepatic tissues | 55 | |
| 10496538155 | Malate | TCA intermediate May be used for gluconeogenesis | 56 | |
| 10496538156 | a-Ketoglutarate | Inhibited by aresnite and *increased ammonia (hyperammonemia related diseases)* | 57 | |
| 10496538157 | Pyruvate Carboxylase | Gluconeogenesis Step 1 enzyme Converts pyruvate to oxaloacetate Requires *biotin* and ATP Allosterically activated by acetyl CoA | 58 | |
| 10496538158 | PEP Carboxykinase | Gluconeogenesis Step 2 enzyme Converts oxaloacetate to phosphoenol pyruvate Requires GTP | 59 | |
| 10496538159 | Fructose-1,6-bisphosphatase | Gluconeogenesis Step 9 enzyme Converts fructose-1,6-bisphospate to fructose-6-phosphate Rate limiting step in gluconeogenesis | 60 | |
| 10496538160 | Glucose-6-phosphatase | Gluconeogenesis Step 11 enzyme Converts glucose-6-phosphate to glucose Final step shared with *glycogenolysis* | 61 | |
| 10496538161 | Cori Cycle | Cycle Lactate is formed by glycolysis in skeletal muscle and is transported to the liver where it is converted back to glucose through *gluconeogenesis* | 62 | |
| 10496538162 | Glycogenin | Protein that serves as a *primer for glycogen synthesis* when glycogen is completely depleted | 63 | |
| 10496538163 | Phosphoglucomutase UDP-glucose Pyrophosphorylase | Enzymes involved in the *synthesis of UDP-glucose* [2] | 64 | |
| 10496538164 | Glycogen Synthase | Enzyme Forms *a(1->4) bond* between glucose residues *Rate-limiting step* Forms the linear portion | 65 | |
| 10496538165 | Branching Enzyme Amylo a(1->4) -> a(1->6) Transglucosidase | Enzyme Forms new *a(1->6) bonds* by transferring 5 to 8 glucosyl residues *Forms the branching portion* | 66 | |
| 10496538166 | Limit Dextrin | 4 glucosyl units in the process of glycogenolysis which signifies the end of the shortening stage Elevated in *debranching enzyme deficiency* | 67 | |
| 10496538167 | Glycogen Phosphorylase | Enzyme Sequential *cleavage of a(1->4)* bonds between the glucosyl residues at the non-reducing ends of the glycogen chain Cleaves until 4 residues are left behind Coenzyme: *pyridoxine* - Vit B6 | 68 | |
| 10496538168 | Phosphoglucomutase Glucose-6-phosphatase | Enzyme Involved in the conversion of glucose-1-P to glucose-6-P *(muscle)* or glucose *(liver)* | 69 | |
| 10496538169 | Galactokinase | Enzyme Phosphorylates galactose to *galactose-1-phosphate* Deficiency leads to *relatively mild symptoms, galactosemia, and cataract formation* | 70 | |
| 10496538170 | Galactose-1-Phosphate Uridyltransferase | Enzyme Converts *galactose-1-phosphate to UDP-galactose* | 71 | |
| 10496538171 | UDP-Hexose-4-Epimerase | Enzyme Converts *UDP-galactose to UDP-glucose* which enters the gluconeogenesis pathway | 72 | |
| 10496538172 | Aldolase B | Enzyme Converts fructose-1-phosphate to DHAP and glyceraldehyde | 73 | |
| 10496538173 | Aldolase Reductase | Enzyme Converts glucose to sorbitol | 74 | |
| 10496538174 | Sorbitol Dehydrogenase | Enzyme Converts sorbitol to fructose | 75 | |
| 10496538175 | Glucuronic Acid | Essential component of glycosaminoglycans Required in the detoxification reaction of insoluble compounds (bilirubin, steroids, morphine) | 76 | |
| 10496538176 | L-gluconolactone Oxidase | Enzyme Used in the synthesis of ascorbic acid in plants and animals | 77 | |
| 10496538177 | NADPH | Reductive biosynthesis of fatty acids and steroids *Glutathione reduction* inside RBCs Cytochrome P450 monooxygenase system Synthesis of *NO* Bactericidal mechanism of *WBC* | 78 | |
| 10496538178 | Linoleic acid 18:2 (9,12) Linolenic acid 18:3 (9,12,15) | Essential Fatty Acids [2] | 79 | |
| 10496538179 | Arachidonic Acid | Fatty acid Precursor of prostaglandins Derived from *linoleic acid* | 80 | |
| 10496538180 | Fatty Acyl CoA Synthetase | Enzyme Activates fatty acid by converting it to Acyl-CoA in the *cytosol* Uses up *2 ATP* | 81 | |
| 10496538181 | Smooth Endoplasmic Reticulum | Organelle where *fatty acids undergo elongation and desaturation*, introducing double bonds up to carbon 9 | 82 | |
| 10496538182 | 1. DHAP from glycolysis 2. Phosphorylation of free glycerol | *Sources of glycerol-3-phosphate* | 83 | |
| 10496538183 | Hormone-sensitive Lipase | Enzyme Catalyzes hydrolysis of of TAG to glycerol + 3 fatty acids Activated by *glucagon and epinephrine* Inhibited by *insulin* | 84 | |
| 10496538184 | Propionyl CoA Carboxylase | Enzyme Converts *propionyl CoA to methylmalonyl CoA* Co-enzyme: *Biotin* | 85 | |
| 10496538185 | Methylmalonyl CoA Mutase | Enzyme Converts *methylmalonyl CoA to succinyl CoA* Co-enzyme: *Methylcobalamin* | 86 | |
| 10496538186 | 7a-Hydroxylase | Enzyme Converts *cholesterol to 7a-hydroxy-cholesterol* Produces primary bile acids, secondary bile acids, bile salts | 87 | |
| 10496538187 | Desmolase | Enzyme Converts *cholesterol to pregnenolone* Produces glucorticoids, mineralocorticoids, testosterone, estradiol, 7-dehydrocholesterol* | 88 | |
| 10496538188 | Thiophorase | Enzyme Breaks down *acetoacetate to acetoacetyl CoA* Found only in peripheral tissue and *NOT IN LIVER* Muscle prefers ketone bodies during fasting because no ATP is needed for its activation | 89 | |
| 10496538189 | Cholic Acid Chenodeoxycholic Acid | Primary Bile Acids | 90 | |
| 10496538190 | Deoxycholic Acid Lithocholic Acid | Secondary Bile Acids | 91 | |
| 10496538191 | Bile Salts | Bile acids that are conjugated to *taurine* and *glycine* | 92 | |
| 10496538192 | Apo A-1 | Apolipoprotein HDL Cofactor of lecithin, cholesterol acyltransferase | 93 | |
| 10496538193 | Apo B-100 | Apolipoprotein Main apoprotein and mediates secretion of *VLDL* | 94 | |
| 10496538194 | Apo B-48 | Apolipoprotein Main apoprotein and mediates secretion of chylomicron | 95 | |
| 10496538195 | Apo C-II | Apolipoprotein Cofactor of lipoprotein lipase - *peripherally cuts TAGs* | 96 | |
| 10496538196 | Apo E | Apolipoprotein Mediates uptake of chylomicron remnants and IDLs | 97 | |
| 10496538197 | Aminotransferase | Step 1: Transamination Enzyme Catalyzes transamination, transfer of a-amino group to a-ketoglutarate forming glutamate Coenzyme: *Pyridoxine* | 98 | |
| 10496538198 | Glutamine Synthetase | Enzyme Catalyzes glutamate combination with 2 ammonia to form glutamine | 99 | |
| 10496538199 | Combination of Amino Acid to Kreb Cycle Metabolite | Alanine-Pyruvate Aspartate-Oxaloacetate Glutamate-a-ketoglutarate | 100 | |
| 10496538200 | Glutamate Dehydrogenase | Step 2: Oxidative Deamination Enzyme Catalyzes oxidative deamination of glutamate to release *free ammonia* Reaction occurs in the liver and kidney | 101 | |
| 10496538201 | Glutaminase | Enzyme Catalyzes glutamine transport and deamination to release ammonia in the liver *(high protein diet)* and kidney *(metabolic acidosis)* | 102 | |
| 10496538202 | ALA Synthase | Enzyme Rate-limiting step Catalyzes glycine + succinyl CoA -> d-aminolevulinic acid Co-factor: *Pyridoxine* | 103 | |
| 10496538203 | ALA Dehydratase | Enzyme Condenses 2 molecules of ALA Contains *zinc* Inhibited by heavy metal ions that replace zinc | 104 | |
| 10496538204 | Ferrochelatase | Enzyme Enhances the introduction of Fe2+ into protoporphyrin IX Inhibited by *lead* | 105 | |
| 10496538205 | Heme Oxygenase | Enzyme Converts heme to biliverdin in the reticuloendothelial cells | 106 | |
| 10496538206 | Ligandin | Protein Intracellular proteins that the bilirubin binds to in the liver | 107 | |
| 10496538207 | Bilirubin Gluconyltransferase | Enzyme Conjugates bilirubin to 2 molecules of glucoronic acid Deficient in *Crigler-Najjar I and II, and Gilbert syndrome* - indirect hyperbilirubinemia | 108 | |
| 10496538208 | 20% | In the brain, ketone bodies can meet up to ____ of the energy requirement | 109 | |
| 10496538209 | PRPP Glutamyl Amidotransferase | Enzyme This is the committed and key regulated step of purine synthesis. It is inhibited by its end product Activated by PRPP Adds first N to PRPP from glutamine. Also uses water and releases glutamate and PP | 110 | |
| 10496538210 | PRPP Synthetase | Enzyme Converts *Ribose 5-Phosphate to PRPP* ATP -> AMP energy requirement | 111 | |
| 10496538211 | Carbamoyl Phosphate Synthetase II | Enzyme Rate limiting enzyme of *de novo pyrimidine synthesis* | 112 | |
| 10496538212 | Ribonucleotide Reductase | Enzyme Multi-subunit enzyme that reduces nucleoside diphosphates to their deoxy forms Redox reactions require *thioredoxin and NADPH* | 113 | |
| 10496538213 | Adenine Phosphoribosyltransferase | Enzyme Salvages adenine by adding it to PRPP to make AMP | 114 | |
| 10496538214 | Thymidylate Synthase | Enzyme Converts *dUMP to thymine* Cofactor: *N5,N10-methylene tetrahydrofolate* Inhibited by *5-fluorouracil* | 115 | |
| 10496538215 | Hypoxanthine-Guanine Phosphoribosyltransferase | Enzyme Purine salvage/degraded to uric acid Hypoxanithine -> IMP Guanine -> GMP | 116 | |
| 10496538216 | mRNA | RNA Largest Copies genetic information from DNA *Template for protein synthesis* MG cap at the 5'-end Poly-A tail at the 3'-end | 117 | |
| 10496538217 | Dihydropteroate Synthetase | Enzyme Not present in eukaryotes Inhibited by *sulfonamides* | 118 | |
| 10496538218 | Dihydrofolate Reductase | Enzyme Inhibited by *trimethoprim* | 119 | |
| 10496538219 | B DNA | Most common form of DNA Right-handed helix with 10 residues per turn | 120 | |
| 10496538220 | rRNA | RNA Most abundant Act as sites for protein synthesis Modified by ribonucleases and endonucleases | 121 | |
| 10496538221 | tRNA | RNA Smallest Adapter molecules that translate the nucleotide sequence of mRNA into specific amino acid Cloverleaf appearance | 122 | |
| 10496538222 | Origin Recognition Complex | Group of proteins that recognize the origin of replication site | 123 | |
| 10496538223 | Helicase | DNA replication enzyme Unwinds the double helix | 124 | |
| 10496538224 | Topoisomerase | DNA replication enzyme Relaxes the super coils Type I (Swivelase) Type II (Gyrase) - inhibited by fluoroquinolone | 125 | |
| 10496538225 | Primase | DNA replication enzyme Synthesizes short segments of *complementary RNA primers* | 126 | |
| 10496538226 | DNA Polymerase III | DNA replication enzyme *Elongates the DNA strand by adding new deoxyribonucleotides* Initiates after primase initiates action | 127 | |
| 10496538227 | DNA Polymerase I | DNA replication enzyme Ribonucleotide removal 5'->3' exonuclease | 128 | |
| 10496538228 | DNA Ligase | DNA replication enzyme Seals the nick by catalyzing the formation of the last phosphodiester bond, requiring hydrolysis of ATP | 129 | |
| 10496538229 | Mismatched Strand | Errors that escape proof reading Mismatch repair Ex. HNPCC | 130 | |
| 10496538230 | Pyrimidine Dimers | Exposure to UV light Nucleotide excision repair Ex. Xeroderma pigmentosum | 131 | |
| 10496538231 | Base Alteration | Spontaneous or deaminating compounds Base excision repair Ex. MUTYH-associated polyposis | 132 | |
| 10496538232 | Double Strand Breaks | Ionizing radiation Free radicals Non-homologous end joining or homologous recombination Ex. SCID, breast cancer | 133 | |
| 10496538233 | Pol I | RNA polymerase Synthesizes: *rRNA* | 134 | |
| 10496538234 | Pol II | RNA polymerase Synthesizes: *mRNA, IncRNA, miRNA, SnRNA* | 135 | |
| 10496538235 | Pol III | RNA polymerase Synthesizes: *tRNA, 5S rRNA* | 136 | |
| 10496538236 | TATA CAAT | DNA promoter sequences in Eukaryotes | 137 | |
| 10496538237 | p-independent Termination | Transcription termination Requires formation of a hairpin loop | 138 | |
| 10496538238 | p-dependent Termination | Transcription termination Requires p protein to release RNA from DNA | 139 |
