Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders

@article{Wiemer1995HumanPT,
  title={Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders},
  author={Erik A. C. Wiemer and William M Nuttley and B L Bertolaet and Xinmin Li and Uta Francke and Margaret J. Wheelock and U K Ann{\'e} and Kenneth R. Johnson and Suresh Subramani},
  journal={The Journal of Cell Biology},
  year={1995},
  volume={130},
  pages={51 - 65}
}
Two peroxisomal targeting signals, PTS1 and PTS2, are involved in the import of proteins into the peroxisome matrix. Human patients with fatal generalized peroxisomal deficiency disorders fall into at least nine genetic complementation groups. Cells from many of these patients are deficient in the import of PTS1-containing proteins, but the causes of the protein-import defect in these patients are unknown. We have cloned and sequenced the human cDNA homologue (PTS1R) of the Pichia pastoris PAS8… CONTINUE READING
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