Absence of phenylalanine hydroxylase
causes

Plasma tyrosine level in Richner-Hanhart
syndrome is

The enzyme acyl-CoA synthase catalyses the conversion of a fatty acid of an active
fatty acid in the presence of

The enzymes of β-oxidation are found in

Free fatty acids are transported in the blood

An important finding of Fabry’s disease
is

A significant feature of Broad Beta disease
is

Amount of phenylacetic acid excreted in
the urine in phenylketonuria is

Neonatal tyrosinemia improves on administration of

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

Richner-Hanhart syndrome is due to
defect in

Salkowski test is performed to detect

The cholesterol molecule is

Carnitine is synthesized from

Molecular formula of cholesterol is

Long chain fatty acids penetrate the inner mitochondrial membrane

Fucosidosis is characterized by

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

A significant feature of Tangier disease is

An important finding in Neimann-Pick
disease is

An important feature of Zellweger ’s
syndrome is

Characteristic finding in Gaucher's disease is

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

Metachromatic leukodystrophy is due to
deficiency of enzyme:

Dietary fats after absorption appear in the circulation as