Zellweger Syndrome

Zellweger Syndrome

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Caption: Brain in rare metabolic disorder, zellweger's syndrome, showing micropolygyria. Zellweger syndrome, is a rare, congenital disorder characterized by the reduction or absence of functional peroxisomes in the cells of an individual. As a result of impaired peroxisome function, tissues and cells can accumulate very long chain fatty acids (VLCFA) and branched chain fatty acids (BCFA) that are normally degraded in peroxisomes. The accumulation of these lipids can impair the normal function of multiple organ systems.

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Keywords: abnormal, brain, cerebrohepatorenal syndrome, condition, congenital abnormality, congenital disorder, cross section, disease, disorder, genetic disorder, gross pathology, gross specimen, leukodystrophies, leukodystrophy, lysosomal structural disorder, medical, pathology, peroxisomal disorder, rare disease, science, slice, unhealthy, zellweger syndrome, zellweger'ss syndrome

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