Question 1
2 out of 2 points
A patient with Niemann-Pick disease will show an increase in intracellular accumulation
of_________?
Selected Sphingomye
Answer: lin
Question 2
2 out of 2 points
The intracellular activity levels of these enzymes will be significantly diminished in a
patient with I-cell disease except___________?
Selected Acid phosphatase
Answer:
Question 3
2 out of 2 points
An eight-month old baby presents to a pediatrician with signs of generalized muscle
weakness, hypotonia, delayed developmental milestones, mental retardation and
appears to have a cherry-red spot on the retina. An evaluation of the baby’s
leukocytes reveals a significant accumulation of gangliosides and the pediatrician
makes a diagnosis of Tay-Sachs disease. This patient will have a reduced activity of
________?
Selected Hexosaminidase A
Answer:
Question 4
2 out of 2 points
In I-cell disease, newly synthesized soluble lysosomal enzymes fail to reach the
lysosomal compartments due to the deficiency of _____?
Selected M6PR
Answer:
Question 5
2 out of 2 points
What amino acid combines with palmitoyl CoA to form sphingosine?
Selected Serine
Answer:
Question 6
2 out of 2 points
In Maple-syrup disease, there is a defective decarboxylation of the α keto groups of
these amino acids except;
Selected Tyrosine
Answer:
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, Question 7
0 out of 2 points
Which of these is not correct about ketogenesis?
Selected The liver carries out ketogenesis but does not use the ketones for
Answer: energy
Question 8
2 out of 2 points
A 22 year old Biology major from Quebec, Canada has been experiencing
progressive muscle weakness, loss of vision in both eyes with a cherry red spot
found on examination of his retina and most recently has been experiencing a
decline in his mental faculties. There was no hepatomegaly or splenomegaly and no
abnormal hematological findings. His fibroblasts showed elevated levels of
gangliosides. You will suspect that the patient is most likely deficient in;
Selected Hexosaminidase A
Answer:
Question 9
2 out of 2 points
Ketogenesis is promoted in humans by an elevated plasma level of __________?
Selected Free fatty Acids
Answer:
Question 10
2 out of 2 points
Which is not true of about antihyperlipidemic drugs?
Selected PCSK-9 inhibitors increase LDL
Answer:
Question 11
2 out of 2 points
In an alcoholic patient who has not eaten food for the past three days, which of these
factors will likely contribute to an elevation in plasma ketone bodies;
Selected All of
Answer: these
Question 12
2 out of 2 points
Niemann-Pick disease is a type of lipid storage disease caused by the genetic
deficiency of ___?
Selected Sphingomyelinase
Answer:
Question 13
2 out of 2 points
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