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1. The glucose-alanine cycle is a method of transferring Alanine
ammonia from the skeletal muscle to the liver. In this
process the non-toxic carrier of ammonia is which of
the following compounds?
2. Which of the following enzymes is correctly paired N-acetylgluta-
with its activator? mate synthatase;
arginine
3. The urea cycle takes place in which of the following the cytosol and
subcellular locations? the mitochondria
4. Which of the following enzymes is regulated by CPS I
N-acetylglutamate (NAGs)?
5. Which of the following enzymes is the regulatory step Carbamoyl phos-
in the urea cycle? phate synthetase I
(CPS I)
6. Which of the following statements is consistent with The urea cycle will
nitrogen transport in the fasted state? be active
7. A comatose infant is brought to the ER. In the course Ornithine tran-
of the examination, plasma ammonia was found to be scarbamoylase
elevated 20-fold over normal values. Urine orotic acid
and uracil were both greater than normal. A defect
of which of the following enzymes is the most likely
diagnosis?
8. Your patient presents with a deficiency of the en- Increased amino
zyme that catalyzes synthesis of N-acetylglutamate acids in the blood
(NAGs). Which of the following would be a conse-
quence of this deficiency in the patient?
9. The cellular uptake of LDL increases intracellular Increases degra-
cholesterol levels. This has which of the following dation of
impacts on de novo cholesterol synthesis? HMG-CoA reduc-
tase
10. Chylomicron
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Dietary cholesterol is primarily transported in the
body by which of the following lipoproteins?
11. Secondary bile salts are generated by bacterial mod- Decrease solubili-
ification of primary bile salts in the intestinal lumen. ty
These modifications have which of the following im-
pacts on bile salt solubility?
12. A 47-year-old male is prescribed a statin (HMG-CoA Decreasing he-
reductase inhibitor) to manage his elevated LDL cho- patic cholesterol
lesterol. What is the initial mechanism of action for biosynthesis
obtaining the desired effect in this man?
13. A deficiency in the LDL receptor will result in an initial Intracellular cho-
decrease in which of the following? lesterol
14. A 45-year-old man has a mild heart attack and a his- Low blood LDLs
tory of vascular disease. He is placed on a Mediter-
ranean diet and statin drug therapy. Which of the
following will be the most likely result of this drug
therapy?
15. A 45-year-old man is found to have an elevated serum Chylomicrons and
triglycerides of > 730mg/dL. He tells you he forgot VLDLs
to fast before his visit and he ate a full breakfast of
bacon and eggs before the labs were drawn. Which of
the following lipoproteins would be the largest con-
tributors to a measurement of plasma triglycerides in
this situation?
16. In the reaction catalyzed by cholesterol ester transfer Cholesterol-ester
protein (CETP), the VLDL particle receives which of
the following compounds from HDL particles?
17. In reverse cholesterol transport HDL interacts with Lecithin choles-
the ABCA1 transporter to move cholesterol from the terol acyltrans-
cell to the HDL. This also requires the activity of which ferase (LCAT)
of the following enzyme?
18.
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HDL particles interact with VLDL particles to ex- Cholesterol es-
change cholesterol ester for triacylglyerols. Which ter transfer protein
of the following enzymes is responsible for this (CETP)
process?
19. Which of the following is a major source of extracel- LDLs
lular cholesterol for human tissues?
20. An individual is suspected of having a deficiency Thiamine
in branched chain keto acid dehydrogenase. Sup-
plementation of which of the following vitamins or
cofactors may help resolve this issue?
21. A 55-year old man presents to his primary care physi- Cysteine
cian with bouts of dizziness and confusion. He is
diagnosed with hyperhomocysteinemia due to a defi-
ciency of cystathionine-ß synthase. In this individual
which of the following amino acids could potentially
become essential?
22. Phenylketonuria can be caused by an enzymatic de- Tetrahydro-
ficiency or it can present due to a deficiency in which biopterin (BH4)
of the following required metabolic cofactors?
23. A 17-year old female presents to her primary care and Conversion of
is diagnosed with a Vitamin B12 (cobalamin) deficien- methyl mal-
cy. In this individual, which of the following reactions onyl-Co A to suc-
would be compromised? cinyl-CoA
24. A 15-year-old female presents to her pediatrician with Tryptophan
concerns over her inability to sleep, increased feel-
ings of depression and generalized fatigue. A full
laboratory work-up is ordered and based on these
findings she is diagnosed with a melatonin deficien-
cy. Based on this information, metabolism of which
of the following amino acids is likely impaired?
25. A 52-year- old male sought medical help complain- Homogentisate
ing about progressive appearance of hyperchromic oxidase
(hyper pigmented) papules on the edge of the sec-