PEX14 encodes an essential component of the peroxisomal import machinery. Peroxisomal biogenesis factor 14 is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. Peroxisomal biogenesis factor 14 also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in PEX14 results in one form of Zellweger syndrome.