… of the genetic phenotype-complementation of peroxisome … 12 CGs of peroxisome-deficient
CHO cell mutants by these … PTS1 proteins are in peroxisomes. (B) Cloning of pathogenic …

… to the second peroxisomal targeting signal (PTS1) is unclear. … biogenesis disorders, PBDs)
due to mutations in genes … as a cross-complementation between the mutation HS3E in the …

… of peroxisome targeting signal 1 (PTS1) cargo protein, be monoubiquitinated and exported
back into the cytosol. Importantly, the mutant … a novel missense mutation in PEX5 responsible …

… is encoded by a single gene and imported into peroxisomes by the PTS1 receptor Pex5 in a
… by genetic phenotype-complementation assay with a peroxisome-defective CHO cell mutant…

… Arabidopsis drp3a and drp3b mutants both display larger and fewer mitochondria, but only
… by the PEX5 (PTS1 proteins) or PEX7 (PTS2 proteins) receptors. Receptor-cargo complexes …

… caused by mutations in genes coding for peroxisomal matrix … PTS1 and PTS2 proteins are
transported to the peroxisome … PEX5(C11K) was also shown to complement the import of PTS1…

… and implementing clinical biochemical genetic testing for peroxisomal disorders, …
Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome …

… peroxisomes are involved in important metabolic pathways and, accordingly, mutations in
genes encoding peroxisomal … This property, together with their capacity to bind PTS1 and …

… X-ALD is caused by mutations in the ABCD1 gene that encodes a peroxisomal transmembrane
… A small number of peroxisomal matrix proteins lack either PTS1 or PTS2 within their …

… shuttles between cytosol and peroxisomes and releases cargo … receptor and cargo release.
Our results reveal the unique mechanism by which PEX5 ferries proteins into peroxisomes…