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Gene Review

PCP1  -  Pcp1p

Saccharomyces cerevisiae S288c

Synonyms: MDM37, Mitochondrial distribution and morphology protein 37, Processing of cytochrome c peroxidase protein 1, RBD1, Rhomboid protein 1, mitochondrial, ...
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Disease relevance of PCP1


High impact information on PCP1


Biological context of PCP1

  • Mutations in the catalytic domain lead to complete loss of Pcp1p function [5].
  • Sequence comparisons show that the protein contains zinc finger motifs and shares weak homologies with the Drosophila couch potato gene, which serves as a transcriptional regulator, indicating that PCP1 activates a silent endogenous sucrose uptake system [6].
  • We have characterized a novel protein, RNA-binding domain-1 (RBD-1), that is involved in ribosome biogenesis [4].
  • Pcp1p overexpression causes chromosome missegregation, multiple mitotic spindle fragments, and multiple abnormal SPB-like structures, a phenotype remarkably similar to that of many human carcinoma lines, which exhibit chromosome and spindle defects, and supernumerary centrosomes [7].

Anatomical context of PCP1

  • Maternal RBD-1 in the fertilized egg disappears immediately after cleavage starts, whereas zygotic RBD-1 first appears in late embryos and is localized in the nucleolus in most cells, although zygotic transcription of pre-rRNA is known to be initiated as early as the one-cell stage [8].

Associations of PCP1 with chemical compounds

  • In this paper, we show that ugo2 mutants are defective in PCP1, a gene encoding a rhomboid-related serine protease [5].
  • One of these cDNAs (PCP1) enabled efficient growth of the mutant yeast strain and mediated uptake of radiolabeled sucrose [6].
  • Disruption of the SPE2 gene encoding S-adenosylmethionine decarboxylase, which catalyzes an essential step in polyamine biosynthesis, upregulated the initial velocity of spermidine uptake in wild-type cells as well as in the polyamine transport-deficient pcp1 mutants [9].

Other interactions of PCP1

  • Our study demonstrates that Pcp1p is required for the processing of Mgm1p and controls normal mitochondrial shape and mtDNA maintenance by producing the 90 kDa form of Mgm1p [5].
  • RBD-1, a nucleolar RNA-binding protein, is essential for Caenorhabditis elegans early development through 18S ribosomal RNA processing [8].
  • RBD-1 is the Caenorhabditis elegans homolog of Mrd1p, which was recently shown to be required for 18S ribosomal RNA (rRNA) processing in yeast [8].

Analytical, diagnostic and therapeutic context of PCP1


  1. Isolation and characterization of Desulfitobacterium frappieri sp. nov., an anaerobic bacterium which reductively dechlorinates pentachlorophenol to 3-chlorophenol. Bouchard, B., Beaudet, R., Villemur, R., McSween, G., Lépine, F., Bisaillon, J.G. Int. J. Syst. Bacteriol. (1996) [Pubmed]
  2. Mitochondrial membrane remodelling regulated by a conserved rhomboid protease. McQuibban, G.A., Saurya, S., Freeman, M. Nature (2003) [Pubmed]
  3. Alternative topogenesis of Mgm1 and mitochondrial morphology depend on ATP and a functional import motor. Herlan, M., Bornhövd, C., Hell, K., Neupert, W., Reichert, A.S. J. Cell Biol. (2004) [Pubmed]
  4. A novel conserved RNA-binding domain protein, RBD-1, is essential for ribosome biogenesis. Björk, P., Baurén, G., Jin, S., Tong, Y.G., Bürglin, T.R., Hellman, U., Wieslander, L. Mol. Biol. Cell (2002) [Pubmed]
  5. Cells lacking Pcp1p/Ugo2p, a rhomboid-like protease required for Mgm1p processing, lose mtDNA and mitochondrial structure in a Dnm1p-dependent manner, but remain competent for mitochondrial fusion. Sesaki, H., Southard, S.M., Hobbs, A.E., Jensen, R.E. Biochem. Biophys. Res. Commun. (2003) [Pubmed]
  6. A novel zinc finger protein encoded by a couch potato homologue from Solanum tuberosum enables a sucrose transport-deficient yeast strain to grow on sucrose. Kühn, C., Frommer, W.B. Mol. Gen. Genet. (1995) [Pubmed]
  7. Pcp1p, an Spc110p-related calmodulin target at the centrosome of the fission yeast Schizosaccharomyces pombe. Flory, M.R., Morphew, M., Joseph, J.D., Means, A.R., Davis, T.N. Cell Growth Differ. (2002) [Pubmed]
  8. RBD-1, a nucleolar RNA-binding protein, is essential for Caenorhabditis elegans early development through 18S ribosomal RNA processing. Saijou, E., Fujiwara, T., Suzaki, T., Inoue, K., Sakamoto, H. Nucleic Acids Res. (2004) [Pubmed]
  9. The spermidine transport system is regulated by ligand inactivation, endocytosis, and by the Npr1p Ser/Thr protein kinase in Saccharomyces cerevisiae. Kaouass, M., Gamache, I., Ramotar, D., Audette, M., Poulin, R. J. Biol. Chem. (1998) [Pubmed]
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