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mhewett's version from 2016-07-18 20:19

Section 55.0 Lipid Metabolism

Question Answer
What is the substrate in fatty acid synthesis? What is the end product?Acetyl CoA; Palmitic acid
Acetyl CoA in the cell cytoplasm, when present in excess, will be metabolized to _____.Malonyl CoA
What enzyme catalyzes the conversion of acetyl CoA to malonyl CoA?Acetyl CoA carboxylase
What is the rate limiting enzyme in fatty acid synthesis?Acetyl CoA carboxylase
How is the activity of acetyl CoA carboxylase effected by insulin? Glucagon? Epinephrine?Activates; Inhibits; Inhibits
How does malonyl CoA effect the activity of carnitine palmitoyltransferase I?Inhibits
What enzyme facilitates the movement of acyl CoA across the inner mitochondrial membrane?Carnitine palmitoyl transferase
What is the fatty acid from which all other fatty acids are produced?Palmitic acid
What enzyme catabolizes palmitic acid?Fatty acyl CoA desaturase
What are the two essential fatty acids?(1) Linolenic acid (2) Linoleic acid
Where does ketogenesis exclusively occur?Liver mitochondria
What are the two most common causes of ketogenesis?(1) Type I diabetes (2) Starvation
What is the rate limiting enzyme in the breakdown of triglycerides (fats)?Hormone sensitive lipase
What are the two products of triglycerides (fats) catabolism by hormone sensitive lipase?(1) Free fatty acids (2) Glycerol
How does insulin effect the activity of hormone sensitive lipase? Epinephrine?Inhibits; Activates
What are the three primary ketoacids?(1) Acetoacetate (2) 3-hydroxybutyrate (3) Acetone
Once in the mitochondrial matrix, acyl CoA is converted into _____ by beta oxidation.Acetyl CoA
In the mitochondrial matrix, what are the two possible pathways for acetyl CoA?(1) TCA (2) Ketogenesis (occurs when there is an over abundance of acetyl CoA)
How does carnitine deficiency effect fatty acid oxidation? Ketone body production? Free fatty acids?Decreases; Decreases; Increases
What are the two most common causes of carnitine deficiency?(1) Prematurity (2) Hemodialysis
memorize

Section 55.5 Eicosanoids

Question Answer
What is the major eicosanoid? Are eicosanoids saturated or unstaturated fatty acids?Arachidonic acid; Unsaturated
What are the three most significant derivative of arachidonic acid?(1) Prostaglandins (2) Thromboxane (3) Leukotrienes
What enzyme catalyzes the removal of arachidonic acid from membrane phospholipids?Phospholipase A2
What enzyme catalyzes the metabolism of arachidonic acid to prostaglandins (and, later, thromboxane)?Cyclooxygenase
What enzyme catalyzes the metabolism of arachidonic acid to leukotrienes?Lipoxygenase
What derivative of arachidonic acid causes vasodilation, exudation, and pain?Prostaglandins
What derivative of arachidonic acid causes platelet aggregation?Thromboxane
What derivative of arachidonic acid causes potent bronchoconstriction?Leukotrienes
memorize

Section 55.6 Fatty Acids

Question Answer
How many carbons compose palmitoleic acid? How many double bonds does it have? Is it saturated, mono-unsaturated, or poly-unsaturated?16; 1; Mono-unsaturated
How many carbons compose oleic acid? How many double bonds does it have? Is it saturated, mono-unsaturated, or poly-unsaturated?18; 1; Mono-unsaturated
How many carbons compose linoleic acid? How many double bonds does it have? Is it saturated, mono-unsaturated, or poly-unsaturated?18; 2
Poly-unsaturated
How many carbons compose linolenic acid? How many double bonds does it have? Is it saturated, mono-unsaturated, or poly-unsaturated?18; 3; Poly-unsaturated
How many carbons compose arachidonic acid? How many double bonds does it have? Is it saturated, mono-unsaturated, or poly-unsaturated?20; 4; Poly-unsaturated
How many carbons compose palmitic acid? How many double bonds does it have? Is it saturated, mono-unsaturated, or poly-unsaturated?16; 0; Saturated
How many carbons compose stearic acid? How many double bonds does it have? Is it saturated, mono-unsaturated, or poly-unsaturated?18
0
Saturated
memorize

Section 55.7 Cholesterol Synthesis

What is the substrate in cholesterol synthesis? What is the end product? Acetly CoA; Cholesterol
What is the rate limiting enzyme for the production of cholesterol? HMG CoA reductase
How is the activity of HMG CoA reductase effected by insulin? Glucagon? Glucocorticoids? Cholesterol? Activates; Inhibits; Inhibits; Inhibits

Section 55.7.1 Cholesterol Functions

What is the precursor for all steroids in the body (e.g. cortisol, sex hormones, etc.)? Cholesterol

Section 55.8 Bile Acids

Because cholesterol cannot be fully metabolized in humans, it must be eliminated with _____. Bile
What part of the small intestine is associated with the reabsorption of bile salts and acids? Ileum

Section 55.9 Clinical Issues: Disorders due to Excess Cholesterol

What is the primary cause of atherosclerosis? What type of cells are characteristically seen in this disease? High LDL-cholesterol; Foam cells (cholesterol-laden macrophages)
What is the most common type of gallstone in worldwide? What are its main components? Mixed stone; (1) Bilirubin (2) Cholesterol

Section 55.10 Lipid Structures

How many fatty acid chains would you expect to find on a triglyceride? Glycolipid? Phospholipid? Sphingolipid? 3; 1; 2; 1
What is the only phospholipid that is antigenic to human? Cardiolipin
What are the two primary components of a glycolipid? (1) Ceramide (2) Sugar(s)
What are the three components of ganglioside (special type of glycolipid)? (1) Ceramide (2) Oligosaccharide (3) N-acetylneuraminic acid (NANA)
What type of lipid composes the bulk of the myelin sheath that insulates nerve fibers of the CNS? Sphingolipids

Section 55.11 Sphingolipidoses

What two sphingolipidoses are associated with cherry red maculas? Which is also associated with hepatosplenomegaly? (1) Niemann-pick (2) Tay-sachs; Niemann-pick
What two sphingolipidoses are most commonly seen in Ashkenazi jews? (1) Gaucher's (2) Tay-sachs
What sphingolipidosis is associated with a complete absence of myelin and early death? Krabbe's disease
What sphingolipidosis is associated with angiokeratomas, reddish-purple rase, kidney failure, and heart failure? What is its pattern of inheritance? Fabry; X-linked recessive
What are the six sphingolipidoses? How are they inherited? (1) Niemann Pick - Autosomal recessive (2) Krabbe's - Autosomal recessive (3) Gaucher's - Autosomal recessive (4) Tay Sachs - Autosomal recessive (5) Metachromatic dystrophy - Autosomal recessive (6) Fabry - X-linked recessive
What sphingolipidosis is associated psychiatric problems? Metachromatic dystrophy
Which of the six sphingolipidoses in not associated with severe CNS dysfunction? What is this condition's pattern of inheritance? Fabry; X-linked recessive
Which of the six sphingolipidoses is associated with a defect in sphingomyelinase? Beta-galactosidase? Niemann-Pick; Krabbe's
Which of the six sphingolipidoses is associated with a defect in Beta-glucosidase? Alpha-galactosidase? Gaucher's; Fabry's
Which of the six sphingolipidoses is associated with a defect in aryl sulfatase? Hexosaminidase? Metachromatic dystrophy; Tay-Sachs

Section 56.0 Digestive Enzymes

What type of cells release pepsinogen? What causes pepsinogen to be cleaved into pepsin? What is the function of pepsin? Chief cells; HCl; Degrades proteins into peptides and free amino acids
What organ produces trypsinogen? What are the two ways be which trypsinogen is converted into trypsin? Pancreas; (1) Enteropeptidase (2) HCl (from stomach)
What is the function of trypsin? Cleaves proteins at the carboxy side of arginine and lysine
What organ produces chemotrypsinogen? What causes chemotrypsinogen to be cleaved into chemotrypsin? Pancreas; Trypsin
What is the function of chemotrypsin? Cleaves proteins at the carboxy side of phenylalanine, tyrosine, tryptophan, or leucine
What organ produces proelastase? What causes proelastase to be cleaved into elastase? What is the function of elastase? Pancreas; Trypsin; Cleaves elastin
What type of cells release lactase? What is the function of lactase? Brush border cells; Cleaves lactose into glactose and glucose

Section 57.0 Amino Acids - Metabolism

Question Answer
What enzyme catalyzes the conversion of phenylalanine to tyrosine? What is its cofactor?Phenylalanine hydroxylase; Tetrahydrobiopterin (THP)
What amino acid is DOPA directly derived from?Tyrosine
What are the three neurotransmitters derived from DOPA?(1) Dopamine (2) Norepinephrine (3) Epinephrine
What are the three primary derivatives of tyrosine?(1) DOPA (2) Thyroid hormone (T3/T4) (3) Homogentisate
What is the only location where norepinephrine is converted into epinephrine?Adrenal medulla
Which of the following is NOT a derivative of tyrosine? A) Melanin B) DOPA C) Thyroid hormone D) Niacin E) None of the aboveD) Niacin
What amino acid is niacin (vitamin B3) derived from?Tryptophan
What two neurotransmitters are derived from tryptophan?(1) Serotonin (2) Melatonin
What amino acid is most associated with tuna and turkey?Tryptophan
What neurotransmitter is derived from glutamate? Is it stimulatory or inhibitory?GABA; Inhibitory
What amino acid is alanine derived from? Tyrosine? Aspartic acid?Glutamate; Phenylalanine; Glutamate
What amino acid is responsible for the formation of heme?Glycine
How does lead effect heme synthesis?Decreases by inhibiting delta ALA dehydratase stoping dALA from becoming porphobilinogen (microcytic anemia)
What amino acid is creatinine derived from? What three parts of the body contain creatine kinase (CK)?Glycine; (1) Heart (2) Skeletal muscle (3) Brain
What two molecules combine to form heme? What enzyme catalyzes the conversion?(1) Iron (2) Protoprophyrin IX; Ferrochelatase
What amino acid is histamine derived from? Nitric oxide?Histidine; Arginine
memorize

Section 57.1 Methylation of Molecules

What molecule is often said to be the great methylator, providing methyl groups to molecules throughout the body? S-adenosyl methionine (SAM)

Section 57.2 Enzyme Disorders

What enzyme is deficient/defective in albinism? Tyrosinase
What is the pattern of inheritance for ocular albinism? Oculocutaneous albinism? X-linked; Autosomal recessive
What enzyme is deficient/defective in phenylketonuria? How does it effect levels of DOPA, norephinephrine, and ephinephrine? Phenylalanine hydroxylase OR Tetrahydrobiopterin (THP); Decreases
What enzyme disorder is characterized by musty odor urine, hypopigmentation, and intellectual disability? What is the treatment? Phenylketonuria; Phenylalanine free diet
What enzyme is deficient/defective in alkaptonuria? Homogenisate oxidase
What enzyme disorder is characterized by ochronosis (pigmentation of connective tissue) and darkly pigment urine? Alkaptonuria
Parkinsonism is due to an inability to convert _____ to _____. DOPA; Dopamine
What enzyme is deficient/defective in maple syrup urine disease? How is it inherited? Alpha-ketoacid dehydrogenase; Autosomal recessive
What are the three branched chain amino acids? What enzyme disorder is characterized by an accumulation of branched chain amino acids? How is it treated? (1) Isoleucine (2) Leucine (3) Valine; Maple syrup urine disease; Diet free of branched chain amino acids
What enzyme is deficient/defective in homocystinuria? Cystathionine synthase
What enzyme disorder is characterized by lens dislocation, increased platelet aggregation, endothelial damage, and mental retardation? Homocystinuria
Cystinuria is an autosomal recessive disorder associated with impaired tubular resorption of what four amino acids? Which of these crystallizes to form a stone? (1) Cysteine (2) Ornithine (3) Arginine (4) Lysine, MNEMONIC: COAL; Cysteine stone
What enzyme disorder is characterized by a defect im membrane transport of trytophan? What vitamin deficiency does it cause? Hartnup disease; Niacin (vitamin B3) deficiency
What are the three Ds of pellagra? What is the primary cause of this disease? (1) Dermatitis (2) Dementia (3) Diarrhea; Niacin (vitamin B3) deficiency
What enzyme catalyzes the conversion of glutamate to aspartic acid? To alanine? Aspartate aminotransferase (AST); Alanine aminotransferase (ALT)
What enzyme catalyzes the conversion of glutamate to GABA? Pyridoxal phosphate (PLP; Vitamin B6)

Section 57.2.1 Enzyme Characteristics and Kinetics

What is the primary goal of enzymes? Lower Gibbs energy (energy needed to bring about a reaction)
_____ are sites, other than the active site, found on enzymes the often result in a conformational change or other change in the effectiveness of an enzyme. Allosteric sites
Enzymes that require cofactors or coenzymes, but have none bound, are called _____. Those with cofactors or coenzymes bound are called _____. Apoenzymes; Holoenzymes
The maximum rate at which an enzyme can produce a product is known as _____. Vmax
_____ is the substrate concentration required for an enzyme to reach one-half of its maximum reaction rate (i.e. Vmax). Michaelis-Menten constant (Km)
The higher the Michaelis-Menten constant (Km) for any particular substrate, the _____ substrate is necessary to help the enzyme achieve half of its maximal speed (Vmax). The higher the Km the _____ the affinity of substrate for enzyme. More; Lower
The lower the Michaelis-Menten constant (Km) for any particular substrate, the _____ substrate is necessary to help the enzyme achieve half of its maximal speed (Vmax). The lower the Km the _____ the affinity of substrate for enzyme. Less; Higher
What are the four types of enzyme inhibitors? (1) Competitive (2) Uncompetitive (3) Non-competitive (4) Mixed
How do competitive inhibitors effect Vmax? Km? Unchanged; Reduced
How do non-competitive inhibitors effect Vmax? Km? Reduced; Unchanged
Are competitive inhibitors influenced by substrate concentration? How about non-competitive inhibitors? Yes; No