| 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 |