Peroxisomes: the neuropathological consequences of peroxisomal dysfunction in the developing brain.

[zellweger syndrome]

Peroxisomes are intracellular organelles that perform vital metabolic functions . They have been extensively studied in the hepatic and renal systems , yet their pivotal roles in facilitating central nervous system patterning and in disease pathogenesis are only recently being firmly established by the neuroscience community . Peroxisomal functions including the break-down of long chain fatty acids , the removal of H 2 O 2 , and the biosynthesis of ether lipids . The build up of long chain fatty acids and H 2 O 2 is detrimental to cellular function , and ether lipids play roles in maintaining cell membrane structure . These findings have major implications for treatments for the full spectrum of peroxisomal disorders . Here , we provide a timely review highlighting the most important data in recent times linking peroxisomal functions to brain formation , and we describe how peroxisomal deficiency and pathway dysfunction results in neurological deficits , the more severe of which result in life changing disabilities and death .