Fats And Fatty Acid Metabolism Set 2

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Biochemistry (Fats And Fatty Acid Metabolism Set 2)

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1. Long chain fatty acids are first activated
to acetyl-CoA in

A) Cytosol

B) Microsomes

C) Nucleus

D) Mitochondria

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2. Richner-Hanhart syndrome is due to
defect in

A) Tyrosinase

B) Phenylalanine hydroxylase

C) Hepatic tyrosine transaminase

D) Fumarylacetoacetate hydrolase

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3. 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

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4. Carnitine is synthesized from

A) Lysine and methionine

B) Glycine and arginine

C) Aspartate and glutamate

D) Proline and hydroxyproline

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5. Neonatal tyrosinemia improves on administration of

A) Thiamin

B) Riboflavin

C) Pyridoxine

D) Ascorbic acid

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6. Dietary fats after absorption appear in the circulation as

A) HDL

B) VLDL

C) LDL

D) Chylomicron

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7. Characteristic finding in Gaucher's disease is

A) Night blindness

B) Renal failure

C) Hepatosplenomegaly

D) Deafness

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8. 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

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9. Fucosidosis is characterized by

A) Muscle spasticity

B) Liver enlargement

C) Skin rash

D) Kidney failure

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10. An important finding of Fabry’s disease
is

A) Skin rash

B) Exophthalmos

C) Hemolytic anemia

D) Mental retardation

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11. Absence of phenylalanine hydroxylase
causes

A) Neonatal tyrosinemia

B) Phenylketonuria

C) Primary hyperoxaluria

D) Albinism

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12. Molecular formula of cholesterol is

A) C27H45OH

B) C29H47OH

C) C29H47OH

D) C23H41OH

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13. Metachromatic leukodystrophy is due to
deficiency of enzyme:

A) α-Fucosidase

B) Arylsulphatase A

C) Ceramidase

D) Hexosaminidase A

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14. 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

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15. Salkowski test is performed to detect

A) Glycerol

B) Cholesterol

C) Fatty acids

D) Vitamin D

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16. 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

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17. An important finding in Neimann-Pick
disease is

A) Leukopenia

B) Cardiac enlargement

C) Corneal opacity

D) Hepatosplenomegaly

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18. 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

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19. Gaucher’s disease is due to deficiency of
the enzyme:

A) Sphingomyelinase

B) Glucocerebrosidase

C) Galactocerbrosidase

D) β-Galactosidase

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20. 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

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21. The enzymes of β-oxidation are found in

A) Mitochondria

B) Cytosol

C) Golgi apparatus

D) Nucleus

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22. Palmitic, oleic or stearic acid ester of cholesterol used in manufacture of cosmetic creams is

A) Elaidic oil

B) Lanoline

C) Spermaceti

D) Chaulmoogric oil

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23. A significant feature of Broad Beta disease
is

A) Hypocholesterolemia

B) Hypotriacylglycerolemia

C) Absence of Apo-D

D) Abnormality of Apo-E

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24. 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

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25. The cholesterol molecule is

A) Benzene derivative

B) Quinoline derivative

C) Steroid

D) Straight chain acid

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Fats And Fatty Acid Metabolism Set 2

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