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This reacts with bilirubin in the liver to form conjugated bilirubin (water soluble) This is the substrate for

gluconeogenesis and for synthesizing triacylglycerol This is derived from glucose and is osmoticalyactive and can damage the lens, schwann cells, and pericytes. This happens to glucose to trap it in cells for further metabolis This is what causes basement membranes of small vessels to render them permeable to protiens causing hylaine a This is formed by a glucose reaction with a terminal amino group, used for measuuing long term glucose concentr Maltose can be broken down into Lactose can be broken down into Sucrose can be broken down into This cleaves the 1,4 linkages in glycogen to release glucose into the blood This is an important component of fiber but cannot be digested in humans These are important components of the extracelular matrix Digestive enzymes cleave {{c1::-glycosidic}} bonds in startch but no {{c1::-glycosidic}} bonds in cellulose This can be oxidized to generate the energy needed by cells in the fasting state These are the two essential fatty acids. These are directly absorbed in the SI and transported to the liver through the portal vein This type of fatty acid is found in triacylglycerols (fat) and structureal lipids Long chain fatty acids require this to move from the cytosol into the mitochondria These are formed by esterification of fatty acids with glycerol Cortico steroids reduce this release from membranes by inactivating phospholipase A2 Surfactain contains a lot of this to decrease surface tension Defects in lysosomal enzymes that degrade this cause acummulation leading to lysosomal storage disease (e.g. Ta This is a precursor for steroid hormones, vitamin D, and bile acids This is formed by action of cyclooxygenase on arachidonic acid This substance promotes platelet aggregation, vasoconstriction, and bronchoconstriction This compound begins with the hydroxylation of arachidonic acid by lipoxygenase This is a strong chemotactic agent for neutrophils and activates neutrophil adhesion molecules for adhesion to en This class of drugs target receptors of this compound and inhibit lipoxygenase, preventing bronchoconstriction in These 3 amino acids are in maple syrup urine disease In PKU, metabolites of this aa accumulate and are neuro toxic These two aa stimulate growth hormone and insulin and are important for growth in children This aa is an inhibitory NT of the spinal cord, is part of the synthesis of heme and is abundant in collagen This aa is a component of glutathione and is deficient in G6PD deficiency These are responsible for kuru and Creutzfeldt-Jacob disease In the SI the pH is ... so weak based drugs are absorbed well Deficiency of this enzyme results in increased peroxidase in RBCs which denatures Hb, forms Heinz bodies This disease process is characterized by thick mucous secretions in the airways, pancreas, and intestines due to an Collagen is made up of the following proteins This disease is characterized by general weakness, bleeding from the gums, loosening of teeth and formation of re This syndrome is characterized by hyperextensible skin and joints, poor wound healing, and cigarette paper scars Patients with this disease have a collagen synthesis deficiency causing a tendency to suffere multiple fractures due

Fetal hemoglobin is made of what two globin subunits Thalassemias present themselves as what type of anemia? In -thalasemmia leads to an excess of what type of chains that can form inclusion bodies Methemoglobin is a form in which is in this form Acquired methemoglobinemia may occur to oxidizing drugs that oxydize hemoglobin such as... What is the for methemoglobinemia? The Bohr effect is the tendency of hemoglobin to release O2 in response to this condition The Bohr effect conversely implies that increasing pH promotes dissociation of what compound, increasing the O2 In sickle cell anemia, sythesis of <sub>2</sub><sub>s2</sub> is due to this defect This compound is a byproduct of glycolysis that reduces O2 affinity for O2 BPG levels are in RBCs of persons in high altitude due to the reduced saturation of Hb This is a derivative of niacin that participates in most oxidation-reduction reactions this compound is covalently attached to enzymes that catalyze carboxylation reactions These are substrate analogues that compete with normal substrate binding to the active site These compete with ethanol for binding sites to alcohol dehydrogenase, causing toxicity. This medication competitivelyl inhibits dihydrofolate reductase, leading to reduced DNA synthesis These rebind reversibly away from the active site forming unreactive enzyme inhibitor and enzyme substrate inhib This irriversible inhibitor acetylates the active site of cyclooxygenase. What effect does it produce? Organophosphates in pesticides irreversibly inhibit this enzyme Which Creatine kinase predominates in skeletal muscle in the brain smooth muscle and lungs in cardi Alkaline phosphatase is a serum marker for Creatine kinase-MM is a serum marker for what disease -Glutamyltransferase (GGT) is increased in what disease Which serum marker is more specific for acute pancreatitis, lipase or amyase? Defects in synthesis of this molecule causes porphyria and sideroblastic anemias This molecule has a 200 fold greater affinity for Hb and produces cherry red discoloration of the skin and organs This pH state causes a right shift of the O2 binding curve A type of glycosylated Hb formed by binding of glycose to the terminal amino group of subunit in HbA This disease is marked by servere hemolytic anemia, autosplenectomy, multiorgan pain due to microvascular occlu This increases the synthesis of HbF and reduces the number of sickle cell crisis This type of thalassemia is mos prevalent in Asian and African populations. Bart's disease occurs with a total absen This type of thalasemia causes an increase in HbA2 and HbF due to the absence of this chain In this syndrome, antibodies are directed against the BM of pulmonary and glomerular capillaries causing hemopt In this disease there is an inherited deficiency of sphingomyelinase causing spingomyelin to accumulatein the lysos Homocystinura is a deficiency of this enzyme causing osteoporosis, dislocated optic lens, and risk for blood clot These types of enzymes add or subtract electrons These enzymes transfer small groups such as: amino, acyl, phosphoryl, one-carbon, sugars These enzymes add water across bonds to cleave them These enzymes add the elements of water, ammonia, or CO2 across a double bond These enzymes make structural rearrangements These enzymes join molecules together

These enzymes use ATP tophosphorylate the substrate and use Mg as a co-factor These enzymes are used in all AA metabolism with B6 as a co-factor These enzynes make a carbon carbon bond using CO2 with Biotin as their co-factor These enzymes join things together without the use of ATP These enzymes join things together with the use of ATP These enzymes cause a loss of hydrogen using a co-factor (NAD, NADH, FAD, FADH...) Neurotoxic venoms of snakes inhibit this enzyme which results in a trasient period of contraction followed by prol Patients who've had a heart attack or stroke are by IV administration of these enzymes to break down fibrin clot When drinking alcohol, people with the 'Asian flush' are lacking which enzyme? This antibiotic inhibits transpeptidases responsible for crosslinking bacterial cell walls, leading to lysis of weakened This antibiotic serves as structural analogs for PABA that prevents formation of folic acid in bacteria

Glucuronic acid Glycerol 3 phosphate Sorbitol Phosphorylation glucose-6-phosphate Glycosylation of sugars Hemoglobin A1C Glucose glucose + galactose glucose + fructose Glycogen phosphorylase Cellulose Glycosaminoglycans (GAGs) and Hyaluronic acid nds in cellulose Fatty acids Linoleic acid and Linolenic acid Short chain and Medium chain fatty acids Long chain fatty acids Carnitine shuttle Triacylglycerols Arachidonic acid Phospholipids, particularly phosphatidylcholine Sphingolipids Cholesterol Prostaglindins Thromboxane 2 LTB4 Antileukotriene drugs Isoleucine, leucine, and valine Phenylalanie Arginine and Histidine Glycine Cysteine Prions Alkaline G6PD Cystic Fibrosis Glycine, Proline, Hydroxyproline and Hydroxylysine Scury is a Vitamin C deficiency Ehlers-Danlos Syndrome Osteogenesis Imperfecta

2 -globins and 2 -globins Hemolytic anemia -chains Oxidized ferric Fe3+ Sulfanilamide, acetaminophen, benzocaine, and sodium nitroprusside Methylene blue Decreased pH Protons from the Hb Replacement of a glutamic acid at position 6 by valine on the subunit 2,3-bisphosphoglycerate (BPG) elevate Nicotine adenine dinucleotide (NAD+) Biotin Competitive inhibitors Methanol and ethylene glycol Methotrexate Noncompetitive inhibitor Asprin reduces the synthesis of prostaglandins and throboxanes Acetylcholinesterase CK-MM in skeletal muscle; CK-BB in the brain smooth muscle and lungs; CK-MB in cardiac muscle Osteoblastic bone disease &amp; obstructive liver disease Duchenne muscular dystrophy Obstructive liver disease and in alcoholics Lipase Heme synthesis Carbon monoxide Acidotic state HbA1c Sickle Cell Anemia Hydroxyurea thalassemia Thalassemia Good Pasture's syndrome Niemann pick disease Cystathionine -synthase Oxidoreductases Transferases Hydrolases Lyases Isomerases Ligases

Kinases Transaminases Carboxylase Synthases Synthetase Dehydrogenase Acetylcholinesterase Tissue plasminogen activator or streptokinase Aldehyde hydehydrogenase Penicillin Sulfanilamides

Liver is the only tissue with glycerol kinase to phosphorylate glycerol for gluconeogenesis Diabetes Mellitus can cause cataracts, neuropathy and retinopathy due to sorbitol

This excludes the brain and RBCs

Phospholipase A2 is used for degradation of phospholipids They are localized in white matter of the CNS

Zileuton, inhibits lipoxygenase. Zafirlukast and montelukast<u>,</u> block leukotriene receptors they are hydrophobic Add tyrosine to their diet

These are infectious proteins formed by a change in 2 structure instead of genetic mutation. The drugs lose their proteons and become non-ionized The CTFR gene causes the receptor to fold wrong. Every third aa is glycine Gly-Pro-X and Gly-X-hydroxyproline It impairs collagen production and structure. Vit C is necessary to hydroxylate proline during synthesis An autosomal dominant disease that affects collagen chains in collagen types I and III aka Brittle Bone disease; type II is most severe

It has a high affinity for O2 They form a tetramer that precipitates in RBCs Ferrous Fe2+ can bind O2 and is reduced back by NADPH-cytochrome b5 reductase a dye that reduces Fe3+ back to Fe2+ Conditions that prevail in metabolically active tissues It is an autosomal recessive disorder BPG drives the oxy-to-deoxy (T R form) conversion of Hb to promote O2 dilivery to the tissues to enhance dissociation of O2 in tissue to compensate for the hypoxia e.g. glycolytic pathway e.g. pyruvate carboxylase The EI complex is inactive High substrate conc reverese comopetitive inhibiton by saturating enzyme with substrate subtrate does not reverse inhibition

Irreversible inhibition of this enzyme leads to accumulation of acetylcholin at the synapses neurologic impairme rdiac muscle

Amylase can also be seen in mumps It is an iron-cotaining porphyrin ring with 100% O2 or hyperbaric treatment promotes unload of O2 to the tissues

HbF inhibits sickling 4 tetramers destroys RBCs in hemoglobin H variant ( chains deficient) Cooley's anemia: no HbA produced; requires regular blood transfusions Fe overload Presents with failure to thrive or learning disorders It prduces Cystathionine, a precursor of cysteine Dehydrogenases, Redutases, Oxidases Kinases, phosphotransferases, aminotransferases Glycosidases, Nucleases, peptidases Decarboxylases, Dehydratases, Hydratases Mutases, Epimerases Synthases and Synthetases

B6 def neuropathy : amytryptaline, cabamazepine No bonds are broken

This breaks down the NT acetylcholine within nerve synapses Causing acetaldehyde vasodilation

ne receptors

e during synthesis

the tissues

apses neurologic impairment