Biochem Final Key things
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Questions and Answers

Match each disease to its disrupted enzyme

Phenylketonuria = Phenylalanine deficiency: infant musty odor Alkaptonuria = Homogentisate Oxidase: Black urine and bones Maple Syrup Disease = Branched chain amino acids (valine + Isoleucine) Tyrosinemia I = Fumarylacetoacetate Hydrolase: Death in 1 year; cabbage body odor

Match each disease to its cause

Tyrosinemia II = Tyrosine Aminotransferase disruption Hyperhomocysteinemia = Mutation in Serine Hydroxymethyltransferase; mutated cystathione-b-synthase Transient Tyrosinemia = Immature hydroxyphenylpyruvate enzymes, Vitamin C deficiency, and Elevated intake of tyrosine and phenylalanine Folate Deficiency = Elevated uracil in DNA; reduced dTTP

Which of the following can donate carbons?

  • Alanine
  • Valine
  • Glycine (correct)
  • Aspartate

    • What is decreased in Hyperhomocysteinemia?

    <p>B12/ B6</p> Signup and view all the answers

    What is not one of the elements needed for purine synthesis

    <p>Valine</p> Signup and view all the answers

    What reaction requires tetrahydrobiopterin?

    <p>Phenylalanine to tyrosine</p> Signup and view all the answers

    Which of the following does not correctly describe the metabolism of serine?

    <p>Generates cystathionine when combined with S-adenosylmethionine</p> Signup and view all the answers

    Which of the following is not needed to generate cysteine?

    <p>Biotin</p> Signup and view all the answers

    Which of the following spontaneously forms a ring structure?

    <p>Glutamate semialdehyde</p> Signup and view all the answers

    Which of the following can generate glycine?

    <p>Threonine</p> Signup and view all the answers

    An increase of which class of amino acids leads to maple syrup urine disease?

    <p>Branched chain amino acids</p> Signup and view all the answers

    Which of the following is not correctly matched?

    <p>Phenylketonuria and black bone</p> Signup and view all the answers

    Which of the following is the most abundant tetrahydrofolate species in the human body?

    <p>N5-methyl-FH4</p> Signup and view all the answers

    Which unique feature is found in Vitamin B12?

    <p>Presence of cobalt</p> Signup and view all the answers

    During a vitamin B12 or folate deficiency, which organ can still recycle Methionine from Homocysteine?

    <p>Liver</p> Signup and view all the answers

    Which of the following is not correctly matched?

    <p>Iron storage and transferrin</p> Signup and view all the answers

    How many nuclei are found in a megakaryocyte that is actively releasing platelets?

    <p>8</p> Signup and view all the answers

    Which of the following terms describes the platelet membrane?

    <p>Canalicular</p> Signup and view all the answers

    Which of the following is not an accurate statement of von Willebrand factor?

    <p>Stabilizes clotting factor VII in the blood</p> Signup and view all the answers

    Match each degradation pathway to its enzymes and diseases

    <p>Phenylalanine = Enzymes: Phe hydroxylase (PAH), homogentisate oxidase; Disease: PKU classic alcaptonuria Tyrosine = Enzymes: Fumarylacetoacetate hydrolase, tyrosine aminotransferase; Diseases: Tyrosinemia I &amp; II Methionine = Enzymes: Cystathionase, Cystathionine- beta-synthase; Diseases: Cystathioninuria, Homocysteinemia Glycine = Glycine transaminase (Gly-&gt; Oxalate); Disease: Oxaluria type 1</p> Signup and view all the answers

    Match each group of amino acids to what they produce

    <p>Pyruvate = Alanine, Cysteine, Glycine, Serine, Threonine, Tryptophan Acetyl-CoA via acetoacetyl- CoA = Leucine, Lysine, Phenylalanine, Tryptophan Acetyl-CoA directly = Isoleucine, Leucine, Threonine, Tryptophan Alpha-ketoglutarate = Arginine, Glutamate, Glutamine, Histidine, Proline</p> Signup and view all the answers

    Match each group of AA to what they produce

    <p>Succinyl- CoA = Isoleucine, Methionine, Threonine, Valine Fumarate = Phenylalanine, Tyrosine Oxaloacetate = Asparagine, Aspartate N/A = N/A</p> Signup and view all the answers

    Which of the following is critical in the activation of platelets?

    <p>Thrombin</p> Signup and view all the answers

    Which of the following is important in the extrinsic clotting cascade and not found in the intrinsic pathway?

    <p>Tissue factor</p> Signup and view all the answers

    SERPIN drugs would not affect which of the following enzymes?

    <p>Factor XIII</p> Signup and view all the answers

    Salvage pathways are important for which of the following?

    <p>Adenine</p> Signup and view all the answers

    Which of the following donates a carbon to help form purines?

    <p>Carbon dioxide</p> Signup and view all the answers

    Which of the following amino acids in its entirety is included in purines?

    <p>Glycine</p> Signup and view all the answers

    Which of the following is formed first during purine and pyrimidine de novo synthesis?

    <p>RNA bases</p> Signup and view all the answers

    Which of the following catalyzes the commitment step towards purine synthesis

    <p>Glutamine PRPP amidotransferase</p> Signup and view all the answers

    Which of the following provides necessary energy for the formation of GMP?

    <p>ATP</p> Signup and view all the answers

    Which of the following is not an allosteric inhibitor of purine synthesis?

    <p>UMP</p> Signup and view all the answers

    Which of the following is the target of allopurinol?

    <p>Xanthine oxidase</p> Signup and view all the answers

    The following can convert adenosine to AMP directly?

    <p>Adenosine kinase</p> Signup and view all the answers

    The following can convert guanine to GMP directly

    <p>Hypoxanthine-guanine phosphoribosyltransferase (HGPRT)</p> Signup and view all the answers

    Deficiency of which of the following can lead to severe combined immunodeficiency?

    <p>Adenosine deaminase</p> Signup and view all the answers

    Which of the following catalyzes the commitment step of pyrimidine synthesis? And where in the cell does the synthesis occur?

    <p>Carbamoyl phosphate synthetase II and in the cytosol</p> Signup and view all the answers

    What is the first pyrimidine generated in denovo synthesis?

    <p>UMP</p> Signup and view all the answers

    Which of the following is an allosteric activator of carbamoyl phosphate synthetase II?

    <p>PRPP</p> Signup and view all the answers

    Which of the following is not a peptide hormone

    <p>Cortisol</p> Signup and view all the answers

    Which of the following will negatively affect the release of growth hormone ONLY from the anterior pituitary?

    <p>Growth hormone releasing inhibitory hormone</p> Signup and view all the answers

    What is the precursor for dopamine

    <p>Tyrosine</p> Signup and view all the answers

    Which of the following is the active form of thyroid hormone?

    <p>T3</p> Signup and view all the answers

    Which of the following can activate thermogenin through norepinephrine?

    <p>Thyroid hormone</p> Signup and view all the answers

    Which of the following is not correctly matched?

    <p>Fetal hemoglobin alpha 2 beta 2</p> Signup and view all the answers

    Which of the following is not a correct statement about hemoglobin S and C?

    <p>Hemoglobin S protects from malaria whereas hemoglobin C does not</p> Signup and view all the answers

    Which of the following terms is associated with normal red blood cell morphology?

    <p>Normochromic</p> Signup and view all the answers

    Which pathway produces the most ATP in the red blood cell

    <p>Glycolysis</p> Signup and view all the answers

    The hexose-monophosphate shunt is analogous to which of the following pathways?

    <p>Pentose Phosphate Pathway</p> Signup and view all the answers

    2, 3 bisphosphoglycerate is generated from which glycolysis intermediate?

    <p>1,3 bisphosphoglycerate</p> Signup and view all the answers

    What is the purpose of cytochrome b5 reductase

    <p>Reduce ferric</p> Signup and view all the answers

    Which of the following is a precursor of heme?

    <p>Glycine</p> Signup and view all the answers

    Zinc can inhibit heme synthesis by incorporating into which compound?

    <p>Protoporphyrinogen IX</p> Signup and view all the answers

    What is the average lifespan of the red blood cell?

    <p>120 days</p> Signup and view all the answers

    Which of the following is not a correct statement about the degradation of bilirubin?

    <p>Yellow sclera associated with hypobilirubinemia</p> Signup and view all the answers

    How is iron transported in the blood?

    <p>As transferrin</p> Signup and view all the answers

    In red blood cell structure, which of the following proteins is a transmembrane protein?

    <p>Band 3 protein</p> Signup and view all the answers

    Which of the following is a negative regulator of platelet activity?

    <p>PGI2</p> Signup and view all the answers

    Which of the following clotting factors is not dependent on Vitamin K?

    <p>XII</p> Signup and view all the answers

    Which of the following is not activated by thrombin?

    <p>Factor IX</p> Signup and view all the answers

    Match each structure to its action

    <p>GPIA = Binds collagen GPIB = Binds von Willebrand factor PKC = Release granules ADP release = Attractant (recruits more platelets)</p> Signup and view all the answers

    Match each item to its action

    <p>GPII A + GPII B = Bind fibrinogen and induce aggregation (inhibited by rattlesnake venom) Stellate cells = Store Vitamin A Alpha actin and myosin (heavy chains) = ATPase activity Alpha actin and myosin (light chains) = Calcium</p> Signup and view all the answers

    Match each structure to its action

    <p>CYP34A = Grapefruit inhibits; metabolizes most drugs CYP2D6 = Codeine, antidepressants, opioids, etc. CYP2E1 = Alcohol, tylenol, vinyl chloride/ Binds protein and DNA CYP1A1 = Enhances aflatoxin B1, fluvoxamine metabolism</p> Signup and view all the answers

    In damaged tissue, what does vonWilibrand bind during platelet adhesion?

    <p>Collagen</p> Signup and view all the answers

    Which of the following is an important attractant for platelets and is released by activated platelet?

    <p>ADP</p> Signup and view all the answers

    Which of the following factors can thrombin directly activate?

    <p>V</p> Signup and view all the answers

    Which of the following blood clotting factors is part of the intrinsic but not extrinsic pathway?

    <p>XI</p> Signup and view all the answers

    Which of the following clotting cascade factors is a direct target for activated protein C?

    <p>VIII</p> Signup and view all the answers

    The amount of which of the following determines the half life of the red blood cell?

    <p>Glucose-6-phosphate dehydrogenase</p> Signup and view all the answers

    What is the reducing agent used by cytochrome b5 reductase?

    <p>NADH</p> Signup and view all the answers

    Which of the following is a precursor for heme synthesis?

    <p>Succinyl-CoA</p> Signup and view all the answers

    Which of the following is the regulated step of heme synthesis?

    <p>delta-aminolevulinic acid synthase</p> Signup and view all the answers

    When breaking down heme, what is the fate of the globin portion of the hemoglobin molecule?

    <p>Precursor for amino acids</p> Signup and view all the answers

    Which of the following cell supports neuron growth and development?

    <p>Astrocyte</p> Signup and view all the answers

    Which of the following is a ciliated cell that secretes cerebrospinal fluid (CSF)?

    <p>Ependymal cell</p> Signup and view all the answers

    Which of the following is another name for growth hormone releasing hormone?

    <p>Somatocrinin</p> Signup and view all the answers

    Which of the following is NOT a trigger for the release of growth hormone?

    <p>Decrease in blood amino acids</p> Signup and view all the answers

    What form of thyroid hormone is stored in the colloid space?

    <p>Both T3 and T4</p> Signup and view all the answers

    What is the fate of RNA synthesis in a cell in the presence of cortisol signaling?

    <p>Decreases synthesis</p> Signup and view all the answers

    What is the stored form of folate in the body?

    <p>5-methyl THF</p> Signup and view all the answers

    What of the following is the most oxidized form of tetrahydrofolate?

    <p>N10-formyl THF</p> Signup and view all the answers

    Which species of THF can donate a methyl group to UMP to generate TMP?

    <p>N5, N10-Methylene THF</p> Signup and view all the answers

    Which of the following can donate carbons to form a purine ring structure?

    <p>N10-formyl THF</p> Signup and view all the answers

    What is the most abundant source of THF in the body?

    <p>Serine metabolism</p> Signup and view all the answers

    In a patient with a Vitamin B12 deficiency, what is the most common cause in the United States?

    <p>Intrinsic factor disorder</p> Signup and view all the answers

    Which of the following does not provide a nitrogen to a purine ring structure?

    <p>Alanine</p> Signup and view all the answers

    What is the activated ribose called?

    <p>5-phosphoribosyl-1-pyrophosphate</p> Signup and view all the answers

    Orotic Aciduria is formed during which of the following synthesis pathways?

    <p>Pyrimidine</p> Signup and view all the answers

    Which of the following characteristics of cytochrome p450 enzymes is correct?

    <p>They all oxidize the substrate on which they act</p> Signup and view all the answers

    Drinking grapefruit juice while taking statins can lead to potentially devastating side effects. This is caused by a component of grapefruit juice doing which of the following?

    <p>Inhibiting the inactivation of statins</p> Signup and view all the answers

    Which of the following metabolizes Aspergillus Aflatoxin B1

    <p>CYP2A1</p> Signup and view all the answers

    Which of the following metabolizes acetaminophen?

    <p>CYP2E1</p> Signup and view all the answers

    Which of the following metabolizes vinyl chloride?

    <p>CYP2E1</p> Signup and view all the answers

    Which of the following metabolizes opioids?

    <p>CYP2D6</p> Signup and view all the answers

    Which of the following is the most abundant in the liver and gut?

    <p>CYP3A4</p> Signup and view all the answers

    Which of the following has ATPase activity?

    <p>Alpha actin</p> Signup and view all the answers

    This molecule blocks actin and myosin from interacting?

    <p>Troponin I</p> Signup and view all the answers

    This is a product that forms spontaneously in creatine metabolism

    <p>Creatinine</p> Signup and view all the answers

    Match each cell to its function

    <p>Hepatocyte = Metabolically necessary Endothelial cells = Leaky Kupffer cells = Macrophages Pit cells = Natural killer cells</p> Signup and view all the answers

    Match each cell to its function

    <p>Tropomyosin = Binds actin in the presence of calcium; makes actin available to interact with myosin Troponin T = Binds tropomyosin Troponin C = Binds calcium Troponin I = Blocks actin and myosin interaction</p> Signup and view all the answers

    Match each item to whether it can get in or out of the blood brain barrier

    <p>Must get in = Essential AA, Essential FA, CO2, H2O, O2 Must not get in or out = Non-essential FA, Non-essential AA, Alanine Must get in = Glucose, Ketone bodies, Insulin, IGF, Vitamine Must not get in or out = Glycine, Proline, GABA</p> Signup and view all the answers

    In the biosynthesis pathways for heme, creatine, and guanine, which one of the following amino acids directly provides carbon atoms that appear in the final product?

    <p>Glycine</p> Signup and view all the answers

    Which of the following is the most important inhibitory neurotransmitter of the CNS?

    <p>GABA</p> Signup and view all the answers

    Which of the following is the most important excitatory neurotransmitter of the CNS?

    <p>Glutamate</p> Signup and view all the answers

    Which of the following must not cross from the blood and into the brain?

    <p>Glycine</p> Signup and view all the answers

    Study Notes

    • Phenylketonuria: Disrupted enzyme - Phenylalanine Hydroxylase
    • Alkaptonuria: Disrupted enzyme - Homogentisate 1,2-Dioxygenase
    • Hyperhomocysteinemia: Decreased - Methionine
    • Maple Syrup Urine Disease: Increased - Branched-Chain Amino Acids
    • Reaction requiring Tetrahydrobiopterin (BH4): Phenylalanine Hydroxylase
    • Vitamin B12 unique feature: Ability to transfer methyl groups
    • Vitamin B12 or Folate deficiency: Liver can still recycle Methionine from Homocysteine
    • Most abundant Tetrahydrofolate species: 5-Methyl-THF

    Platelet Structure and Function

    • Megakaryocyte features: Multiple nuclei
    • Platelet membrane feature: Phospholipid Bilayer
    • Inaccurate statement about von Willebrand factor: Not involved in platelet adhesion
    • Critical for platelet activation: Collagen
    • Extrinsic clotting cascade component: Tissue Factor
    • SERPIN drugs do not affect: Plasminogen Activator Inhibitor-1

    Amino Acid Metabolism

    • Cysteine generation: Serine and Homocysteine
    • Glycine generation: Choline, Serine, and Threonine
    • Purine synthesis donors: Glycine, Aspartate, Glutamine, and CO2
    • Inosine Monophosphate (IMP) synthesis: First step in purine synthesis

    Coagulation Cascade

    • Unique feature of Vitamin K: Essential for clotting factor synthesis
    • Thrombin-activated factors: Factor VIII, Factor XI, and Factor V
    • Intrinsic pathway component: Factor IX
    • Activated Protein C target: Factor Va
    • Platelet attractant: Thromboxane A2

    Red Blood Cell Structure and Function

    • Red Blood Cell membrane protein: Glycophorin
    • Negative regulator of platelet activity: Prostacyclin
    • Hemoglobin S and C incorrect statement: Not associated with sickle cell anemia
    • Red Blood Cell morphology term: Discocyte
    • Most ATP-producing pathway: Glycolysis

    Heme Synthesis

    • Heme precursor: Succinyl-CoA and Glycine
    • Zinc incorporation compound: Protoporphyrin IX
    • Red Blood Cell lifespan: 120 days
    • Bilirubin degradation statement: Not degraded by the liver
    • Iron transportation protein: Transferrin
    • Cytochrome b5 reductase function: Reduces methemoglobin

    Neurobiology

    • Neuron growth and development supporter: Astrocytes
    • Cerebrospinal fluid (CSF) secreter: Choroid Plexus
    • Growth Hormone Releasing Hormone (GHRH) alternative name: Growth Hormone Releasing Factor (GHRF)
    • Non-trigger for Growth Hormone release: High blood glucose

    Thyroid Hormone and Cortisol

    • Stored Thyroid Hormone form: Triiodothyronine (T3)
    • Cortisol signaling effect: Inhibits RNA synthesis

    Folate Metabolism

    • Stored Folate form: Methyl-THF
    • Most oxidized form of THF: 10-Formyl-THF
    • Methyl donor species: 5-Methyl-THF
    • Vitamin B12 deficiency common cause: Inadequate intrinsic factor production
    • Non-nitrogen donor to purine ring: Aspartate
    • Activated ribose: Phosphoribosyl Pyrophosphate (PRPP)

    Drug Metabolism

    • Grapefruit juice interaction: Inhibits CYP3A4
    • Aspergillus Aflatoxin B1 metabolizer: CYP3A4
    • Acetaminophen metabolizer: CYP2E1
    • Vinyl chloride metabolizer: CYP2E1
    • Opioids metabolizer: CYP2D6
    • Most abundant enzyme in liver and gut: CYP3A4
    • ATPase activity exhibitor: CYP3A4

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