What is the name of the enzyme deficiency that leads to the accumulation of very long-chain fatty acids in the brain, resulting in a neurodegenerative disorder?

A Acyl-CoA dehydrogenase
B Peroxisomal thiolase ✓
C Sterol carrier protein
D Carnitine palmitoyltransferase

Explanation

The deficiency of peroxisomal thiolase leads to the accumulation of very long-chain fatty acids in the brain, resulting in a neurodegenerative disorder known as Zellweger syndrome.

Source

Scriver's OMMBID
Enzymes in Disease Biochemistry Peroxisomal Disorders Hard MEDICAL Confidence: high