Peroxisomal abnormalities in metabolic diseases

Abstract

Two congenital metabolic disorders that can be thought of as preoxisomal diseases may provide clues to the function of peroxisomes in mammalian cells. One, acatalasemia, is an enzymatic defect in mice that have been selectively bred for this trait. The second, the cerebrohepatorenal syndrome of Zellweger, is a rare familial human malady characterized by severe muscle weakness, hepatomegaly, cerebral, renal, facial and skeletal abnormalities and death within 6 mth. 27 references are cited.