Article
Hepatic dysfunction in peroxisomal disorders.
Biochimica et biophysica acta - 1 May 2016
Baes Myriam, Van Veldhoven Paul P
Abstract excerpt
The peroxisomal compartment in hepatocytes hosts several essential metabolic conversions. These are defective in peroxisomal disorders that are either caused by failure to import the enzymes in the organelle or by mutations in the enzymes or in transporters needed to transfer the substrates across the peroxisomal membrane. Hepatic pathology is one of the cardinal features in disorders of peroxisome biogenesis and...
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