FINAL COACHING – BIOCHEM 2022 d.

 liver

1. A social worker refers a 2-year-old child to the 6. The ability of rod cells in the eye to respond to light
physician because of suspected child abuse. On and transmit that response to the optic nerve requires
physical examination, the child has blue sclerae and that the 11-cis form of vitamin A be attached to which
diminished hearing in both ears, and radiographs of of the following proteins?
the child’s extremities show subacute healing long Select one:
bone fractures. The child is diagnosed with a. cGMP phosphodiesterase
osteogenesis imperfecta. This disorder results from a b. Na+ channel 
defect in the synthesis of which of the following c. rhodopsin
proteins? d. scotopsin
Select one:
a. Transthyretin  7. You are treating an 11-year-old female type 1 diabetic
b. Calcitonin patient who forgot to eat after she self-administered
c. Spectrin her afternoon insulin. Blood work demonstrates that
d. Collagen her serum glucose level is 1 mM (18mg/dL). Which of
the following statements correctly defines the
2. A male infant is diagnosed with phenylketonuria metabolic situation in this patient?
(PKU) on newborn screening. Which of the following Select one:
amino acids must be present in his diet to prevent a a. a reversal of the muscle hexokinase reaction converts glucose 6-
negative nitrogen balance? phosphate to glucose for oxidation in glycolysis
Select one: b. hepatic glycogen releases glucose in response to insulin-
a. alanine mediated dephosphorylation of glycogen phosphorylase 
b. cysteine  c. hepatic glycogen releases glucose through a reversal of the
c. glutamine glycogen synthase reaction
d. tyrosine d. phosphorylation of hepatic glycogen synthase prevents
glucose incorporation into glycogen
3. You are treating a 30-year-old patient for acute
hepatitis and progressive chronic liver disease. Your 8. You are examining a pediatric patient brought to you
patient also shows parkinsonian tremors, diminished by his parents because they are disturbed by his
facial muscle movement, and choreoathetosis. In progressive mood changes and apparent hightened
taking the patient history you learn that he has anxiety. Physical examination shows pellegra-like skin
become progressively more depressed and paranoid. eruptions and signs of cerebellar ataxia that include
Which of the following disorders most likely explains unsteady gait and uncoordinated eye movements.
the signs and symptoms observed in this patient? Serum and urinalysis show significant aminoacidemia
Select one: and aciduria. These signs and symptoms are most
a. aceruloplasminemia  likely due to which of the following disorders?
b. GRACILE syndrome Select one:
c. hemochromatosis a. alkaptonuria
d. Wilson disease b. glycine decarboxylase deficiency (GCC deficiency) 
c. Hartnup disorder
4. A 6-year-old boy has a long history of a hereditary d. maple syrup urine disease
bleeding disorder characterized by spontaneous
nontraumatic hemorrhages into joint spaces, skeletal 9. Hemoglobin is nonenzymatically glycosylated as
muscle, and mucous membranes. Laboratory studies glucose enters erythrocytes. Because erythrocytes
reveal a normal prothrombin time, elevated partial routinely die and are replaced, the level of
thromboplastin time, very low factor VIII, normal factor glycosylated hemoglobin is a good measure of the
X, normal factor XI, and normal platelet aggregation length of time glucose levels were elevated. Diabetic
studies with ristocetin. Which of the following is the patients are most familiar with this process because
most likely diagnosis? they routinely measure for which of the following?
Select one: Select one:
a. Christmas disease a. HbA1c
b. hemophilia A  b. HbA2
Hemophilia A is a genetic disorder characterized by very low c. HbC 
levels of factor VIII, elevated partial thromboplastin time, d. HbG
normal prothrombin time, normal platelet aggregation with e. HbS
ristocetin, and spontaneous hemorrhages into joints, soft
tissues, and mucosal surfaces. Integ 10. You are carrying out experiments to study DNA
c. hemophilia B replication in a cell line derived from a breast cancer
d. Rosenthal syndrome tumor. You discover that the replication process does
not completely copy all of each chromosome.
5. The active hormonal form of vitamin D requires Examination of the process using electron microscopy
several enzymatic steps. The final step in the indicates that replication ceases near to where the
synthesis of 1,25-dihydroxycholecalciferol occurs in DNA strands are attached to the chromatin scaffold.
which of the following tissues? Given these observations, which of the following
Select one: activities is most likely to be defective in these cells?
a. bone  Select one:
b. intestine a. DNA ligase
c. kidney b. helicase
c. polymerase-α  16. Blocking the actions of which of the following
d. topoisomerase hormones would be expected to lead to the greatest
level of appetite suppression?
11. Accumulating evidence indicates that the nonsteroidal Select one:
anti-infl ammatory (NSAID) aspirin is eff ective in the a. adiponectin
control of numerous chronic conditions such as b. ghrelin
atherosclerosis. The principal cardiovascular benefit c. GLP-1 
from aspirin is due to its ability to reduce the d. obestatin
incidence and severity of thrombotic episodes. The
anticoagulant eff ect of aspirin occurs through its 17. A 30-year-old man seeks help because he lost weight
ability to inhibit which of the following activities? and feels full after eating only a small amount of food.
Select one: He is diagnosed with a delay in gastric emptying.
a. cyclooxygenase Which of the following hormones has at physiological
b. fibrin cross-linking by factor XIIIa  levels the strongest effect in inhibiting gastric
c. thrombin binding to activated platelets emptying?
d. von Willebrand factor Select one:
a. cholecystokinin
12. Select the one of the following statements that is NOT b. gastrin
CORRECT. c. glucose-dependent insulinotropic peptide 
Select one: d. motilin
a. Buffers of human tissue include bicarbonate, proteins, and e. pancreatic polypeptide
orthophosphate.
b. A weak acid or a weak base exhibits its greatest buffering 18. Which of the following conditions is caused by a
capacity when the pH is equal to its pKa plus or minus one pH trinucleotide repeat?
unit. Select one:
c. The isoelectric pH (pI) of lysine can be calculated using the a. Hemophilia A
formula (pK2 + pK3)/2.  b. Prader-Willi syndrome 
d. The mobility of a monofunctional weak acid in a direct c. Fragile X syndrome
current electrical field reaches its maximum when the pH d. Angelman syndrome
of its surrounding environment is equal to its pKa.
19. Which of the following vitamins is critically important
13. You are studying the replication characteristics of a in the overall process of nitrogen transfer from α-
cell line in culture. You find that the DNA in these cells amino acids to α-keto acids?
fragments easily and does not replicate well. Analysis Select one:
of components of the replication machinery and a. riboflavin
associated compounds shows that the levels of b. thiamine
spermidine and spermine are significantly reduced c. vitamin B6 
compared to normal cells. A deficiency in which of the The dominant reactions involved in removing amino acid
following enzymes could best explain the nitrogen from the body are known as transaminations.
observations made with these cells? Transaminations involve moving an α-amino group from a
Select one: donor α-amino acid to the keto carbon of an acceptor α-keto
a. cystathionine β -synthase acid. These reversible reactions are catalyzed by a group of
b. glutamate dehydrogenase intracellular enzymes known as aminotransferases, which
c. methionine synthase  generally employ covalently bound pyridoxal phosphate as a
d. ornithine decarboxylase cofactor.
d. vitamin B12
14. A 58-year-old man visits your office and complains
about impotence. Upon questioning you learn that he 20. A 7-month-old boy has failed to gain weight normally.
is capable of erections, but that they do not last as His diet consists of a diluted milk formula. His weight
long and that they are of lesser strength than and length are below normal for his age, and he
previously. Which of the following neurotransmitters is appears emaciated. His temperature is below normal,
primarily responsible for dilation of the penile artery and his pulse is slow. Which of the following is the
during erections? most likely cause of his condition?
Select one: Select one:
a. acetylcholine a. deficiency of growth hormone 
b. epinephrine  b. marasmus
c. GABA c. hypothyroidism
d. nitric oxide d. kwashiorkor

15. Overproduction of which of the following hormones by 21. Hemin is heme with oxidized ferric (Fe3+) iron. This
a neoplasm is best diagnosed by an increase in compound is known to interfere with the synthesis of
urinary excretion of vanillylmandelic acid? the porphyrins and heme. Which of the following
Select one: enzymes is inhibited by the presence of hemin?
a. ACTH Select one:
b. aldosterone  a. ALA dehydratase 
c. calcitonin b. ALA synthase
d. epinephrine c. ferrochelatase
d. PBG deaminase
 22. Fatty acid metabolism within the brain is not a Excess production of PAF production may be involved in the
significant pathway for the generation of ATP. morbidity associated with toxic shock syndrome and strokes.
However, this does not mean that fatty acid d. prostaglandin E2
metabolism is not a useful pathway in brain. Which of
the following would be expected to be found at 27. You are examining a 5-year-old male child who has
elevated levels in brain tissues following the been exhibiting a tendency to bite his lower lip to the
metabolism of free fatty acids? point of bleeding as well as chewing on his fingertips.
Select one: Physical examination demonstrates swollen tender
a. acetate joints. These symptoms and lab results are indicative
b. alanine  of a deficiency in HGPRT. Given this enzyme
c. ceramide deficiency, which of the following compounds would
d. citrate be expected to be elevated in the serum of this
e. sphingosine patient?
Select one:
23. An 18-year-old boy works in the circus as a a. bilirubin 
contortionist because of his hypermobile joints that b. xanthine
allow for abnormal flexibility. He also has increased c. glycine
elasticity of his skin and bruises easily. This is a d. orotate
typical presentation of Ehlers-Danlos syndrome,
resulting from a defect in collagen. Ehlers-Danlos type 28. The quaternary structure of a given protein is defined
VI results from a lysyl oxidase deficiency. Which of by which of the following?
the following processes will therefore be impaired in Select one:
this disorder? a. structure resulting from the interactions between multiple
Select one: polypeptide chains
a. Transcription b. ordered organization of secondary structures within the protein
b. Glycosylation c. ordered organization of secondary structures within the protein 
c. Hydroxylation  d. overall structure resulting from association of domains within the
d. Covalent cross-linkage protein

24. Which of the following metabolic processes is 29. You are examining the thermodynamically stable
promoted by insulin? structures of proteins. In particular you are studying
Select one: the α-helix and β-sheet conformations that form in the
a. catabolism of muscle protein study proteins. These conformations correspond to
b. catabolism of muscle protein  which of the following?
c. gluconeogenesis Select one:
d. glycogenesis a. native conformation
b. primary structure
25. You are studying a cell line derived from a liver tumor. c. secondary structure
Th is cell line expresses a mutated form of glycerol d. tertiary structure 
kinase. Which of the following lipid classes is most e. quaternary structure
likely to be found at reduced levels in this cell line due
to this gene defect? 30. Hemoglobin and myoglobin are proteins composed
Select one: primarily of which of the following types of secondary
a. cholesterol structures?
b. fatty acids Select one:
c. triglycerides  a. amide bond
Triglycerides are composed of a backbone of glycerol to which b. disulfide bond
3 fatty acids are esterified. One of the pathways to triglyceride c. α -helix 
synthesis in hepatocytes is initiated by the phosphorylation of Both hemoglobin and myoglobin structure are determined by
glycerol via the action of glycerol kinase. Therefore, a defect in the high degree of α -helices present in these proteins. Indeed,
glycerol kinase activity in these cells would result in reduced most of the amino acids in hemoglobin form α -helixes,
capacity to synthesize triglycerides. connected by short nonhelical segments.
d. sphingolipids d. β -pleated sheet

26. Hypersensitive individuals have IgE to specific 31. A 39-year-old woman reports headaches, weakness
antigens (eg, pollen, bee venom) on the surface of and fatigue, and frequent urination over the past
their leukocytes (monocytes, macrophages, several weeks. Physical examination reveals diastolic
basophils, eosinophils). When these individuals are hypertension. Laboratory findings include
challenged with antigen, the antigen–IgE complexes hypokalemia and reduced renin levels. Which of the
induce synthesis and release of which of the following following is the most likely cause of these various
physiologically potent lipids? findings?
Select one: Select one:
a. arachidonic acid a. Conn syndrome
b. leukotriene B4 b. diabetes insipidus 
c. platelet-activating factor (PAF)  c. diabetes mellitus
Platelet-activating factor (PAF) is a unique complex lipid of the d. pheochromocytoma
plasmalogen family. PAF functions in hypersensitivity, acute
infl ammatory reactions, and anaphylactic shock by increased 32. Although most cases of hypertension are “essential,”
vasopermeability, vasodilation, and bronchoconstriction. meaning the underlying disorder is unknown, about
 10% of cases are “secondary hypertension” due to a and there is no significant past history or family
specific, usually treatable cause. Which of the history. Laboratory studies show an elevated fasting
following conditions can lead to secondary blood glucose (11 mmol/L: 198 mg/dL) and a 24-hour
hypertension? urinary cortisol of 2276 nmol/24 h. These signs and
Select one: symptoms are most likely the result of which of the
a. adrenal insufficiency following?
b. estrogen deficiency  Select one:
c. hyperparathyroidism a. Addison disease
d. renal artery stenosis b. Cushing syndrome 
Cushing syndrome describes the clinical consequences of
33. Renin is released from the kidneys in response to a chronic exposure to excess glucocorticoid irrespective of the
drop in blood pressure. The function of renin is to underlying cause. Cushing syndrome may mimic common
generate angiotensin I, which is accomplishes by conditions such as obesity, poorly controlled diabetes, and
which of the following mechanisms? hypertension, which progress over time and often coexist in
Select one: patients with metabolic syndrome. The characteristic features
a. interaction with a steroid receptor in the cell nucleus of Cushing syndrome are psychiatric disturbances (depression,
b. proteolysis of antidiuretic hormone (ADH, vasopressin) mania, and psychoses), central obesity, hypertension,
c. proteolytic cleavage of the precursor in blood  diabetes, moon-shaped face, thin fragile skin, easy bruising,
The intrarenal baroreceptor system is a key mechanism for and purple striae (stretch marks). In addition, Cushing
regulating renin secretion. A drop in pressure results in the syndrome patients manifest with gonadal dysfunction that is
release of renin from the juxtaglomerular cells of the kidneys. characteristic of hyperandrogenism with excess body and
The only function for renin is to cleave a 10-amino-acid peptide facial hair (hirsutism) and acne. The most common symptoms
from the N-terminal end of angiotensinogen circulating in the that lead to a diagnosis of Cushing syndrome are weight gain,
blood. This proteolytic reaction generates angiotensin I. depression, subjective muscle weakness, and headache.
d. stabilization of angiotensin I mRNA c. Graves disease
d. hyperthyroidism
34. The omega-3 class of polyunsaturated fatty acids
(PUFAs) exerts potent effects on neural development, 38. A 32-year-old woman is diagnosed with hypertension,
cardiovascular function, and inflammation. The most hypernatremia, hypokalemia, and alkalosis.
active omega-3 PUFAs are DHA and EPA. These Measurements of plasma glucocorticoid levels show
PUFA are precursors for which of the following class them to be within the normal range; however, renin
of biologically active lipids? and angiotensin II levels are suppressed. Ultrasound
Select one: indicates the possible existence of an adrenal cortical
a. gangliosides mass. These symptoms are likely due to excess
b. leukotrienes production of which of the following hormones?
c. prostaglandins  Select one:
d. resolvins a. aldosterone
b. androstenedione
35. Altered plasma renin levels can occur in both normal c. dehydroepiandrosterone (DHEA) 
and pathologic conditions. Which of the following d. estradiol
states is associated with a decrease in plasma renin e. testosterone
levels?
Select one: 39. A newly wed 23-year-old woman and her 28-year-old
a. heart failure husband are evaluated for infertility. They have been
b. primary aldosteronism unable to conceive a child despite regular intercourse
c. renal artery stenosis for the past 12 months. The first step of this couple’s
d. salt restriction  infertility workup is to determine whether ovulation
occurs regularly. Which of the following hormones is
36. Administration of which of the following hormones is directly sponsible for ovulation?
most likely to produce a positive nitrogen balance? Select one:
Select one: a. estradiol
a. insulin  b. estriol
Positive nitrogen balance refers to the situation where the rate c. follicle-stimulating hormone (FSH) 
of nitrogen incorporation into macromolecules exceeds the d. luteinizing hormone (LH)
nitrogen excretion rate in an individual. Positive nitrogen
balance is most often associated with increased protein 40. A 35-year-old weightlifter who has been injecting
synthesis. One of the major growth factor effects of insulin is testosterone for muscle mass augmentation is
increased protein synthesis which would most likely be evaluated for sterility and found to have an extremely
associated with positive nitrogen balance. low sperm count. Which of the following is an effect of
b. cortisol testosterone and contributes to the mentioned
c. epinephrine sterility?
d. glucagon Select one:
a. activation of inhibin
37. A 45-year-old woman is being examined by her b. feedback activation of leptin
physician because she is concerned about her weight c. feedback inhibition of GnRH 
gain, fatigue, muscle weakness, and thinning hair. Testosterone directly inhibits the secretion of GnRH from the
Physical examination shows a “buffalo hump,” facial hypothalamus, which affects secretion of LH and FSH and
hirsutism, and red striae in abdominal and underarm consequently secretion of testosterone. To initiate
areas. She indicates that she is not on any medication spermatogenesis, both FSH and testosterone are necessary.
 To maintain spermatogenesis after puberty, extremely high mitochondrial uncoupling activity of UCP1. This uncoupling
concentrations of testosterone seem to be required. releases the mitochondrial proton gradient as heat.
Systemically administered testosterone does not raise the c. leptin
androgen level in the testes to as great a degree and it d. adiponectin
additionally inhibits LH secretion. Consequently, the net effect
is generally a decrease in sperm count. 45. Cholestyramine is a drug used to reduce serum
d. inhibition of seminal prostaglandins cholesterol. It functions through binding bile salts and
thereby, interferes with normal enterohepatic
41. You are treating a 54-year-old man with extreme circulation of bile salts in the feces. Use of this drug
abdominal pain. Ultrasonic examination shows would be associated with increased fecal amounts of
enlargement of the pancreas with lobular structures which of the following compounds?
clearly visible. Biopsy of the tissue indicates the Select one:
patient has a glucagonoma. Which of the following a. bilirubin
laboratory fi ndings would also be expected in this b. cholesterol
patient? c. deoxycholate 
Select one: Cholestyramine is a drug belonging to the bile-binding resin
a. decreased (< 6%) hemoglobin A1c class of cholesterol-lowering drugs. These drugs function by
b. decreased serum concentration of free fatty acids binding bile acids in the gut, thereby, preventing their reuptake.
c. hypoinsulinemia Inhibition of bile acid uptake into the enterohepatic circulation
d. increased (350-400 mg/dL) serum glucose concentration  will result in increased bile salt concentrations in the feces,
A glucagonoma is a tumor associated with the α -cells of the including deoxycholate, a primary bile salt. Integ
pancreas that secrete glucagon. Enhanced secretion of d. glucuronate
glucagon would lead to an increase in hepatic
gluconeogenesis and glycogen breakdown. The glucose would 46. Which of the following, associated with obesity, is
then be transported to the blood (resulting in hyperglycemia) most highly correlated to the development of the
preferentially over being oxidized due to the glucagon- metabolic syndrome?
mediated inhibition of the glycolytic enzyme, PFK1. Select one:
42. A 13-year-old adolescent boy with type 1 diabetes a. elevated serum HDL
has a serum glucose concentration is 540 mg/dL. b. elevated serum total cholesterol 
Several hours following IV administration of insulin, c. hyperglycemia
his serum glucose concentration decreases to 200 d. insulin resistance
mg/dL. Which of the following mechanisms best
explains the effect of the insulin signaling pathway on 47. Which of the following is involved in the transport of
the GLUT4 transporter in this decrease resulting in newly synthesized triglycerides from the liver to
the reduced serum glucose? adipose tissue?
Select one: Select one:
a. it activates GLUT4 by a cAMP-dependent mechanism a. chylomicrons 
b. it increases GLUT4 mRNA  b. HDL
c. it promotes GLUT4 mobilization to the plasma membrane c. LDL
d. it induces a conformational change in GLUT4 d. VLDL

43. Addition of which of the following compounds would 48. Which one of following statements about the fed and
be expected to result in the greatest increase in fasting metabolic states is correct?
insulin resistance in adipocytes in culture under Select one:
conditions of hyperglycemia and hyperinsulinemia? a. In the fasting state glucagon acts to increase the activity of
Select one: lipoprotein lipase in adipose tissue.
a. fructose b. In the fasting state, glucagon acts to increase the synthesis of
b. galactose glycogen from glucose. 
c. glucosamine c. In the fed state insulin acts to increase the breakdown of
d. glycine  glycogen to maintain blood glucose.
d. In the fed state there is decreased secretion of insulin in
44. You are carrying out experiments designed to response to increased glucose in the portal blood.
increase energy expenditure as a therapeutic means
to treat obesity. You are testing various compounds in 49. A 58-year-old man with a 30-year history of heavy
experimental animals and find one that appears to drinking presents with confusion, unstable gait, and
satisfy the goals of the experiment in that mice given nystagmus. On the Mini Mental State Examination, he
the compound have a significant increase in scores 21/30, and a diagnosis of Wernicke-Korsakoff
thermogenic activity in their adipose tissue. Th is syndrome is made. Which of the patient’s enzymes is
compound is most likely activating or mimicking the working at a reduced activity level due to the patient’s
activity of which of the following? condition?
Select one: Select one:
a. UCP1 a. Transaldolase 
b. adipose triglyceride lipase  b. Phosphopentose isomerase
Uncoupling protein 1 (UCP1) is a mitochondrial protein of c. Transketolase
brown adipose tissue (BAT) involved in the process of adaptive d. Phosphopentose epimerase
thermogenesis. In response to hormonal stimulation of BAT,
there is an increase in fatty acid release from triglycerides. The 50. A mother has brought her 3-month-old baby to the
fatty acids enter the mitochondria and activate the pediatrician and indicates that the infant is lethargic
and has poor suckling and seems uninterested in
 eating. In addition, the mother notes that the baby’s
diapers often smell like burnt sugar. This infant likely
has a defect in which of the following enzymes?
Select one:
a. branched-chain α-keto acid dehydrogenase
b. cystathionine synthase
c. glycine cleavage complex (GCC)
d. Phosphopentose epimerase 
e. phenylalanine hydroxylase