What condition is characterized by very long fatty acid chain deficiency?

The condition characterized by very long-chain fatty acid deficiency is Zellweger syndrome. This autosomal recessive disorder is part of a group of peroxisomal disorders that arise due to a defect in the peroxisome biogenesis, leading to impaired metabolism of very long-chain fatty acids, among other functions.

In Zellweger syndrome, the deficiency of enzymes necessary for the degradation of very long-chain fatty acids causes these fatty acids to accumulate in various tissues, particularly affecting the brain, liver, and kidneys. This accumulation leads to neurological impairment and other multiorgan system issues associated with the syndrome.

Understanding this condition highlights the importance of peroxisomes in lipid metabolism and how their dysfunction can result in significant health consequences, particularly in infantile cases. Other disorders mentioned, while they have distinct pathophysiologies, do not primarily involve the deficiency of very long-chain fatty acids.