Fats and Fatty Acid Metabolism - Mock Test 45

Question 441

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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Question 442

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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Question 443

Richner-Hanhart syndrome is due to defect in?

A) Tyrosinase B) Phenylalanine hydroxylase C) Hepatic tyrosine transaminase D) Fumarylacetoacetate hydrolase

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Question 444

Absence of phenylalanine hydroxylase causes?

A) Neonatal tyrosinemia B) Phenylketonuria C) Primary hyperoxaluria D) Albinism

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Question 445

Neonatal tyrosinemia improves on administration of?

A) Thiamin B) Riboflavin C) Pyridoxine D) Ascorbic acid

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Question 446

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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Question 447

Metachromatic leukodystrophy is due to deficiency of enzyme:?

A) α-Fucosidase B) Arylsulphatase A C) Ceramidase D) Hexosaminidase A

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Question 448

Fucosidosis is characterized by?

A) Muscle spasticity B) Liver enlargement C) Skin rash D) Kidney failure

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Question 449

An important finding in Neimann?

A) Leukopenia B) Cardiac enlargement C) Corneal opacity D) Hepatosplenomegaly

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Question 450

Character ist ic f ind ing in Gaucher ’s disease is?

A) Night blindness B) Renal failure C) Hepatosplenomegaly D) Deafness

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