Pink-eyed dilution protein controls the processing of tyrosinase

Mol Biol Cell. 2002 Jun;13(6):1953-64. doi: 10.1091/mbc.02-02-0022.

Abstract

The processing of tyrosinase, which catalyzes the limiting reaction in melanin synthesis, was investigated in melan-p1 melanocytes, which are null at the p locus. Endoglycosidase H digestion showed that a significant fraction of tyrosinase was retained in the endoplasmic reticulum. This retention could be rescued either by transfection of melan-p1 cells with an epitope-tagged wild-type p transcript or by treatment with either bafilomycin A1 or ammonium chloride. We found that the endoplasmic reticulum contains a significant amount of p protein, thus supporting a role for p within this compartment. Using immunofluoresence, we showed that most mature full-length tyrosinase in melan-p1 cells was located in the perinuclear area near the Golgi, in contrast to its punctate melanosomal pattern in wild-type melanocytes. Expression of p in melan-p1 cells restored tyrosinase to melanosomes. Triton X-114 phase separation revealed that an increased amount of tyrosinase was proteolyzed in melan-p1 cells compared with wild-type melanocytes. The proteolyzed tyrosinase was no longer membrane bound, but remained enzymatically active and a large proportion was secreted into the culture medium of melan-p1 cells. We conclude that p regulates posttranslational processing of tyrosinase, and hypopigmentation in melan-p1 cells is the result of altered tyrosinase processing and trafficking.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Carrier Proteins*
  • Cell Fractionation / methods
  • Cell Line
  • Cytoplasmic Granules / physiology
  • Cytoplasmic Granules / ultrastructure
  • Endoplasmic Reticulum / physiology
  • Endoplasmic Reticulum / ultrastructure
  • Melanocytes / physiology
  • Membrane Proteins / genetics
  • Membrane Proteins / physiology*
  • Mice
  • Monophenol Monooxygenase / genetics*
  • Monophenol Monooxygenase / metabolism
  • Pigmentation / physiology*
  • Polymerase Chain Reaction
  • Recombinant Proteins / metabolism
  • Transfection

Substances

  • Carrier Proteins
  • Membrane Proteins
  • Recombinant Proteins
  • P protein, mouse
  • Monophenol Monooxygenase