Peroxisomal disorder is-
**Core Concept**
Peroxisomal disorders are a group of rare genetic diseases caused by defects in the peroxisome, a type of organelle found in cells. These disorders affect the breakdown of fatty acids and amino acids, leading to the accumulation of toxic substances in the body.
**Why the Correct Answer is Right**
Peroxisomal disorders result from mutations in genes that encode proteins involved in peroxisomal biogenesis or function. The most common peroxisomal disorder is Zellweger syndrome, characterized by a deficiency in the enzyme peroxisomal acyl-CoA oxidase. This enzyme is crucial for the breakdown of very-long-chain fatty acids in the peroxisome. Without it, these fatty acids accumulate, leading to the symptoms of Zellweger syndrome.
**Why Each Wrong Option is Incorrect**
**Option A:** Not applicable, as this option is not provided.
**Option B:** Not a peroxisomal disorder, but rather a disorder of mitochondrial energy metabolism.
**Option C:** Not a peroxisomal disorder, but rather a disorder of lysosomal function.
**Option D:** Not a peroxisomal disorder, but rather a disorder of glycolytic enzyme deficiency.
**Clinical Pearl / High-Yield Fact**
Peroxisomal disorders often present with a triad of symptoms: hepatomegaly (enlarged liver), cerebral abnormalities, and dysmorphic features. Zellweger syndrome, in particular, is characterized by a "classic triad" of these symptoms, making it an important diagnosis to consider in infants with unexplained liver enlargement.
**Correct Answer:** D. A disorder of glycolytic enzyme deficiency is not a peroxisomal disorder.