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FATS AND FATTY ACID METABOLISM- Part 2
See all quizzes of FATS AND FATTY ACID METABOLISM- Part 2 here:
1 Free fatty acids are transported in the
blood
(A) Combined with albumin
(B) Combined with fatty acid binding protein
(C) Combined with β -lipoprotein
(D) In unbound free salts
2. Long chain fatty acids are first activated
to acetyl-CoA in
(A) Cytosol (B) Microsomes
(C) Nucleus (D) Mitochondria
3. The enzyme acyl-CoA synthase catalyses
the conversion of a fatty acid of an active
fatty acid in the presence of
(A) AMP (B) ADP
(C) ATP (D) GTP
4. Carnitine is synthesized from
(A) Lysine and methionine
(B) Glycine and arginine
(C) Aspartate and glutamate
(D) Proline and hydroxyproline
5. The enzymes of β-oxidation are found in
(A) Mitochondria (B) Cytosol
(C) Golgi apparatus (D) Nucleus
6. Long chain fatty acids penetrate the inner
mitochondrial membrane
(A) Freely
(B) As acyl-CoA derivative
(C) As carnitine derivative
(D) Requiring Na dependent carrier
7. An important feature of Zellweger’s
syndrome is
(A) Hypoglycemia
(B) Accumulation of phytanic acid in tissues
(C) Skin eruptions
(D) Accumulation of C26-C38 polyenoic acid in
brain tissues
8. An important finding of Fabry’s disease
is
(A) Skin rash (B) Exophthalmos
(C) Hemolytic anemia (D) Mental retardation
9. Gaucher’s disease is due to deficiency of
the enzyme:
(A) Sphingomyelinase
(B) Glucocerebrosidase
(C) Galactocerbrosidase
(D) β-Galactosidase
10. Characteristic finding in Gaucher’s
disease is
(A) Night blindness
(B) Renal failure
(C) Hepatosplenomegaly
(D) Deafness
11. An important finding in Neimann-Pick
disease is
(A) Leukopenia
(B) Cardiac enlargement
(C) Corneal opacity
(D) Hepatosplenomegaly
12. Fucosidosis is characterized by
(A) Muscle spasticity (B) Liver enlargement
(C) Skin rash (D) Kidney failure
13. Metachromatic leukodystrophy is due to
deficiency of enzyme:
(A) α-Fucosidase (B) Arylsulphatase A
(C) Ceramidase (D) Hexosaminidase A
14. A significant feature of Tangier disease is
(A) Impairment of chylomicron formation
(B) Hypotriacylglycerolmia
(C) Absence of Apo-C-II
(D) Absence of Apo-C-I
15. A significant feature of Broad Beta disease
is
(A) Hypocholesterolemia
(B) Hypotriacylglycerolemia
(C) Absence of Apo-D
(D) Abnormality of Apo-E
16. Neonatal tyrosinemia improves on administration
of
(A) Thiamin (B) Riboflavin
(C) Pyridoxine (D) Ascorbic acid
17. Absence of phenylalanine hydroxylase
causes
(A) Neonatal tyrosinemia
(B) Phenylketonuria
(C) Primary hyperoxaluria
(D) Albinism
18. Richner-Hanhart syndrome is due to
defect in
(A) Tyrosinase
(B) Phenylalanine hydroxylase
(C) Hepatic tyrosine transaminase
(D) Fumarylacetoacetate hydrolase
19. Plasma tyrosine level in Richner-Hanhart
syndrome is
(A) 1–2 mg/dL (B) 2–3 mg/dL
(C) 4–5 mg/dL (D) 8–10 mg/dL
20. Amount of phenylacetic acid excreted in
the urine in phenylketonuria is
(A) 100–200 mg/dL (B) 200–280 mg/dL
(C) 290–550 mg/dL (D) 600–750 mg/dL
21. Tyrosinosis is due to defect in the enzyme:
(A) Fumarylacetoacetate hydrolase
(B) p-Hydroxyphenylpyruvate hydroxylase
(C) Tyrosine transaminase
(D) Tyrosine hydroxylase
22. An important finding in Histidinemia is
(A) Impairment of conversion of α-Glutamate to
α-ketoglutarate
(B) Speech defect
(C) Decreased urinary histidine level
(D) Patients can not be treated by diet
23. An important finding in glycinuria is
(A) Excess excretion of oxalate in the urine
(B) Deficiency of enzyme glycinase
(C) Significantly increased serum glycine level
(D) Defect in renal tubular reabsorption of glycine
24. Increased urinary indole acetic acid is
diagnostic of
(A) Maple syrup urine disease
(B) Hartnup disease
(C) Homocystinuia
(D) Phenylketonuria
25. In glycinuria daily urinary excretion of
glycine ranges from
(A) 100–200 mg (B) 300–500 mg
(C) 600–1000 mg (D) 1100–1400 mg
26. An inborn error, maple syrup urine
disease is due to deficiency of the enzyme:
(A) Isovaleryl-CoAhydrogenase
(B) Phenylalnine hydroxylase
(C) Adenosyl transferase
(D) α-Ketoacid decarboxylase
27. Maple syrup urine disease becomes
evident in extra uterine life by the end of
(A) First week (B) Second week
(C) Third week (D) Fourth week
28. Alkaptonuria occurs due to deficiency of
the enzyme:
(A) Maleylacetoacetate isomerase
(B) Homogentisate oxidase
(C) p-Hydroxyphenylpyruvate hydroxylase
(D) Fumarylacetoacetate hydrolase
29. An important feature of maple syrup
urine disease is
(A) Patient can not be treated by dietary
regulation
(B) Without treatment death, of patient may occur
by the end of second year of life
(C) Blood levels of leucine, isoleucine and serine
are increased
(D) Excessive brain damage
30. Ochronosis is an important finding of
(A) Tyrosinemia
(B) Tyrosinosis
(C) Alkaptonuria
(D) Richner Hanhart syndrome
