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Zellweger Syndrome

Zellweger Syndrome

C017/4562

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BIOPHOTO ASSOCIATES / SCIENCE PHOTO LIBRARY BIOPHOTO ASSOCIATES / SCIENCE PHOTO LIBRARY

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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