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Biochemistry Worksheet

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mhewett's version from 2016-07-21 14:30

Section

Do M2 and M4 receptors increase or decrease cAMP levels? Decrease
Does cAMP increase or decrease hormone sensitive lipase activity? Increase
What effect do coffee and tea have on cAMP levels? Increase
What enzyme degrades cAMP? Phosphodiesterase
What enzyme forms cAMP? Adenylyl cyclase (adenylate cyclase)
Does insulin activate hormone sensitive lipase? No
Does TSH activate hormone sensitive lipase? Yes
Does epinephrine activate hormone sensitive lipase? Yes
What enzyme cleaves fatty acids off of triglycerides? Hormone sensitive lipase
What is the primary source of VLDL? Fatty acids (triglycerides)
What enzyme allows transfer of acyl CoA across the inner mitochondrial membrane? Carnitine palmitoyl transferase I and II
What effect does malonyl CoA have on carnitine palmitoyl transferase I? Inhibits
From what molecule is malonyl CoA originally derived? Acetyl CoA
What does malonyl CoA eventually become? Palmitate
What is the importance of palmitate production? It is the 1st fatty acid produced to yield all other fatty acids
What enzyme converts acetyl CoA to malonyl CoA? Acetyl CoA carboxylase
What enzyme controls the rate-limiting step of lipogenesis? Acetyl CoA carboxylase
What enzyme converts acetoacetyl acid to HMG-CoA? HMG-CoA Synthase
What enzyme converts HMG CoA to mevalonate in cholesterol synthesis? HMG CoA reductase
What does mevalonate and squalene eventually become? Cholesterol
Does cholesterol production occur in the mitochondrion or cytoplasm of cells? Cytoplasm
What can catalyze the conversion of pepsinogen to pepsin? Hydrogen ions or pepsin
Where is pepsinogen produced? Stomach
Where is enteropeptidase produced? Duodenal mucosa
What can catalyze the conversion of trypsinogen to trypsin? Enteropeptidase or hydrogen ions
What catalyzes chymotrypsinogen, proelastase, and procarboxypeptidases A and B to their active forms? Enteropeptidase
What does pepsin cleave? Proteins
What and where do trypsin, chymotrypsin, and procarboxypeptidases A and B cleave? Proteins, on carboxy side of their respective special sites
What does elastase cleave? Elastin
What are the products of lactase catalysis of lactose? Glucose and galactose
Phenylalanine hydroxylase converts phenylalanine to what? Tryosine
What are the 4 major end-products of tyrosine metabolism? (1) Thyroid hormones (2) Melanin (3) Epinephrine (4) Homogentisate
What functional group is added to norepinephrine to produce epinephrine? Methyl
What is the only place in which epinephrine is made? Adrenal medulla
What enzyme converts tyrosine to DOPA in melanocytes? Tyrosinase
What vitamin is necessary for conversion of dopamine to norepinephrine? Vitamin C
What is pyridoxal phosphate? Vitamin B6
What are the 3 major clinically-significant products of tryptophan? (1) Serotonin (2) Melatonin (3) Niacin
To what neurotransmitter can glutamate be converted? GABA
Is GABA an inhibitory or stimulatory neurotransmitter? Inhibitory
What are the 3 major clinical significant products of glycine? (1) Creatine phosphate (2) Creatine (3) Hemoglobin
What enzyme converts glycine to delta-aminolevulinic acid? Delta-aminolevulinic acid synthase
What enzyme converts delta-aminolevulinic acid to porphyrobilinogen? ALA dehydrase
What 2 enzymes of hemoglobin synthesis does lead inhibit? (1) ALA dehydrase (2) Ferrochelatase
What enzyme converts protoporphyrinogen IX and Fe to heme? Ferrochelatase
Is porphyrinobilinogen a porphyrinogen? No
What functional group is added to glycine and arginine to create creatine? Methyl
What enzyme converts creatine to creatine phosphate? Creatine kinase
What molecule serves as a small reservoir of high energy phosphate for making ATP from ADP? Creatine phosphate
From what is histamine created? Histidine
What is the primary methyl group carrier/transporter? SAM (s-adenosyl methionine)
After methyl group delivery, what does SAM become? SAH (s-adenosyl homocysteine)
What enzyme is deficient in albinism? Tyrosinase
What are 2 possible ways to PKU can occur? (1) Phenylalanine hydroxylase deficiency (2) THP deficiency
What is the triad of PKU? (1) Musty-odored urine (2) Hypopigmentation (3) Mental retardation
What is the enzyme deficiency of alkaptonuria? Homogentisate oxidase
What is the triad for alkaptonuria? (1) Arthritis (2) Dark urine (3) Ochronosis
What is the enzyme deficiency in maple syrup urine disease? Alpha-ketoacid dehydrogenase
What accumulates in maple syrup urine disease? Branched chain amino acids (leucine, isoleucine, valine)
What are the 2 features of maple syrup urine disease? (1) Urine smells like maple syrup (2) MR
What is the enzyme deficiency in homocystinuria? Cystathionine synthase
What is the triad for homocystinuria? (1) Mental retardation (2) Len dislocation (3) Increased coronary artery disease
What is the treatment for homocystinuria? (1) Decreased methionine diet (2) High, high B6 (3) B12 and folate
Why is B6 a treatment for homocystinuria? It increases activity of any cystathionine synthase available
What disorder is marked by a defect in membrane transport of dibasic animo acids? Cystinuria
What is the clinical outcome of cystinuria? Cystine urolithiasis
What is the disorder marked by a defect in membrane transport of tryptophan? Hartnup disease
Which enzyme functions to aid in hepatic glucose "capture" after meal? Glucokinase
What enzyme - gluco- or hexokinase - is always present? Hexokinase
What are the components of maltose? Glucose and glucose
What enzyme converts fructose to fructose-1-PO4? Fructokinase
What disorder is characterized by a defect in fructokinase? Fructosuria
What 3 reactions does aldolase B catalyze? (1) Fructose-1,6,-PO4 to glyceraldehyde (2) fructose-1-PO4 to glyceraldehyde (3) fructose-1-PO4 to dihydroxyacetone
Which is benign: fructosuria or fructose intolerance? Fructosuria
What are the 2 major clnical features of fructose intolerance? (1) Liver failure (2) Hypoglycemia
What is the disease associated with deficient galactose-1-P uridylyl transferase? Galactosemia
What are the 3 primary clinical findings in galactosemia? (1) MR (2) Liver failure (3) Physical stunting
What disorder is characterized by a lack of glucose-6-phosphatase? Type I Glycogen Storage Disease (von Gierke)
What disorder is characterized by a lack of glucosidase? Type II Glycogen Storage Disease (Pompe)
Is glucose-6-phosphatase in muscle? No
Does cAMP inhibit or activate glycogen phosphorylase? Activate
Does cAMP inhibit or activate glycogen synthase? Inhibit
What enzyme is primary in converting glycogen to glucose-6-PO4? Glycogen phosphorylase
What enzyme deficiency exists in Cori's Disease? Branching enzyme
What is the triad for Von Gierke? (1) Hepatomegaly (2) Enlarged kidneys (3) Hypoglycemia
What are the 5 major clinical findings with Pompe's? (1) Hepatomegaly (2) Cardiomegaly (3) Heart Failure (4) Muscle hypotonia (5) Damage to most other organs
What enzyme converts G-6-P in liver and kidney to glucose? Glucose-6-phosphatase
What is the enzyme deficiency in McArdle's? Skeletal muscle phosphorylase
What is the clinical finding in Cori's? Hepatomegaly
What 2 disorders are marked by mutant L iduronidase, albeit 2 different mutations? (1) Hurler (2) Scheie
What 2 mucopolysaccharidoses have corneal clouding? (1) Hurler (2) Scheie
What 2 mucopolysaccharidoses have mental retardation? (1) Hurler (2) Hunter
What mucopolysaccharidosis is marked by a defect in iduronate sulfatase? Hunter
What 2 sphingolipidoses are marked by hepatomegaly and splenomegaly? (1) Niemann-Pick (2) Gaucher's
What 1 sphingolipidosis is not marked by mental retardation? Fabry's
What 2 sphingolipidoses are particularly more common in Ashkenazi Jews? (1) Gaucher's (2) Tay-Sachs
What 2 sphingolipidoses have cherry red maculas? (1) Niemann-Pick (2) Tay-Sachs
What sphingolipidosis is marked by angiokeratomas? Fabry's
What sphingolipidosis has a defect in sphingomyelinase? Niemann-Pick
What accumulates in Niemann-Pick? Sphingomyelin
What sphingolipidosis has a defect in aryl sulfatase? Metachromatic Leukodystrophy
What accumulates in metachromatic leukodystrophy? Sulfatide
What enzyme defect exists in Gaucher's? Beta-glucosidase
What enzyme defect exists in Krabbe? Beta-galactosidase
What accumulates in Gaucher's and Krabbe's? Cerebroside
What enzyme is defective in Tay-Sachs? Hexosaminidase
What accumulates in Tay-Sachs? Ganglioside
What sphingolipidosis is marked by a defect in alpha-galactosidase? Fabry's
What accumulates in Fabry's? Globoside
What does excess heme become? Hemin
What accumulates in acute intermittent porphyria? Porphobilinogen
What accumulates in congenital erythropoietic porphyria? Hydroxymethylbilinogen
What accumulates in cutanea tarda? Uroporphyrinogen III
What accumulates in coproporphyria? Coproporphyrinogen
What porphyria does not yield photosensitivity? Acute intermittent
What do excess porphyrinogens become that ultimately catalyzes the formation of oxygen radicals? Porphyrins
What are the fat-soluble vitamins? A, D, E, and K
What fat-soluble vitamin is critical to maintaining normal epithelium? A
Night blindness and various skin problems are a direct result of deficiency of what vitamin? A
What is the most toxic of all vitamins? D
What vitamin deficiency yields rickets and osteomalacia? D
What vitamin can slow the progression of Alzheimer's disease? E
What is the primary source for vitamin K? Normal bacterial flora in gut
What clotting factors' carboxylation and maturation are dependent upon Vitamin K? II, VII, IX, and X
Why do many elderly have vitamin K deficient-associated bruising? Low gut flora
What vitamin is plentiful in both tomatoes and potato skins? C
What are the 3 functions of vitamin C? (1) Hydroxylation of proline and lysine for collagen synthesis (to ensure maintenance of connective tissue) (2) Acts as Anti-oxidant (3) Cofactor for norepinephrine synthesis
What is another term for vitamin B1? Thiamine
What are the 5 disorders that may result from thiamine deficiency? (1) Wet beriberi (2) Dry beriberi (3) Wernicke's encephalopathy (4) Korsakoff's psychosis (5) Infantile deficiency
What is the primary clinical manifestation of wet beriberi? Cardiomyopathy (dilated)
What is another term for vitamin B2? Riboflavin
Cheilosis and glossitis are classic features of what vitamin deficiency? B2
What water-soluble vitamin is toxic at high doses? B6
What vitamin becomes depleted with the administration of isoniazid? B6
What vitamin aids in some aspect of all amino acid reactions? B6
What are the 2 disorders that result from vitamin B12 deficiency? (1) Macrocytic anemia (2) Neuropathy
What parasite causes vitamin B12 deficiency? Diphyllobothrium latum (the fish tapeworm)
What is the triad of clinical features of pellagra? (1) Diarrhea (2) Dementia (3) Dermatitis
What vitamin is a treatment for hyperlipidemia? B3
What is another term for vitamin B3? Niacin
What are the 4 disorders associated with folic acid deficiency? (1) Macrocytic anemia (2) Colitis (3) Glossitis (4) Neural tube defects
What enzyme in glycolysis converts fructose-6-P to fructose-1,6-P? Phosphofructokinase I (PFK I)
What enzyme in glycolysis acts to shunt off extra fructose-6-P to convert it to fructose-2,6-P? Phosphofructokinase II (PFK II)
What does fructose-2,6-P do to phosphofructokinase I? Activates it
What enzyme in gluconeogenesis converts fructose-1,6-P to fructose-6-P? Fructose-1,6-bisphosphatase
What is the Krebs Cycle substrate that is moved to the cytoplasm to allow for gluconeogenesis? Malate
What are the three ketone bodies? (1) Acetoacetate (2) 3-hydroxybutyrate (3) Acetone
Where in the cell are ketone bodies formed? Mitochondrion
What molecule is a by-product of triglycerides that acts as the initial substrate for ketone body formation in the mitochondrion? Acyl-CoA
What enzyme converts PRPP to 5-phosphoribosyl amine in purine synthesis? Amidotransferase
What is the rate-limiting enzyme in purine synthesis? Amidotransferase
What are the three other terms for the citric acid cycle? (1) Krebs Cycle (2) Tricarboxylic acid cycle (3) TCA Cycle
What are the 3 primary activators of pyruvate dehydrogenase? (1) CoA (2) NAD (3) ADP
What enzyme catalyzes the conversion of pyruvate to acetyl CoA? Pyruvate dehydrogenase
What hormones act to increase amino acid uptake? (1) Insulin (2) Glucagon (in liver) (3) Cortisol (in liver)
What hormone acts to increase glucose uptake? Insulin (into muscle and adipocytes)
What hormone acts to decrease glucose uptake? (1) Growth hormone (and, therefore, causes hyperglycemia) (2) Cortisol (in muscle and adipocytes)
What hormone increases gluconeogenesis and glycogenesis? Cortisol (in liver)
What hormones act to increase lipolysis? (1) Glucagon (in liver) (2) Growth hormone (3) Cortisol (plus epi)
What hormone works to increase both glycogenolysis and gluconeogenesis? Glucagon
What are the pyrimidines? (1) Uracil (2) Cytosine (3) Thymine
What is the term for the region of DNA in bacteria that encodes a single protein? Cistron
What eukaryotic RNA polymerase serves to make mRNA? Polymerase II
What are the 3 major steps of eukaryotic RNA processing? (1) Addition of 5'-cap (2) Addition of poly-A tail (3) Intron excision with splicing exons together
What enzyme serves to make the RNA primer necessary for DNA replication? Primase
The lagging strand is comprised of multiple fragments that are connected together; what are these fragments? Okazaki fragments