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  Domain Name: TPR
Tetratricopeptide repeat domain; typically contains 34 amino acids [WLF]-X(2)-[LIM]-[GAS]-X(2)-[YLF]-X(8)-[ASE]-X(3)-[FYL]-X(2)-[ASL]-X(4)-[PKE] is the consensus sequence; found in a variety of organisms including bacteria, cyanobacteria, yeast, fungi, plants, and humans in various subcellular locations; involved in a variety of functions including protein-protein interactions, but common features in the interaction partners have not been defined; involved in chaperone, cell-cycle, transciption, and protein transport complexes; the number of TPR motifs varies among proteins (1,3-11,13 15,16,19); 5-6 tandem repeats generate a right-handed helical structure with an amphipathic channel that is thought to accomodate an alpha-helix of a target protein; it has been proposed that TPR proteins preferably interact with WD-40 repeat proteins, but in many instances several TPR-proteins seem to aggregate to multi-protein complexes; examples of TPR-proteins include, Cdc16p, Cdc23p and Cdc27p components of the cyclosome/APC, the Pex5p/Pas10p receptor for peroxisomal targeting signals, the Tom70p co-receptor for mitochondrial targeting signals, Ser/Thr phosphatase 5C and the p110 subunit of O-GlcNAc transferase; three copies of the repeat are present here
No pairwise interactions are available for this conserved domain.

Total Mutations Found: 66
Total Disease Mutations Found: 31
This domain occurred 109 times on human genes (206 proteins).



  ASPHYXIATING THORACIC DYSTROPHY 4
  BARDET-BIEDL SYNDROME 4
  BONE MARROW FAILURE, FAMILIAL
  CILIARY DYSKINESIA, PRIMARY, 28, WITH SITUS INVERSUS
  CILIARY DYSKINESIA, PRIMARY, 28, WITHOUT SITUS INVERSUS
  DEFICIENCY
  FETAL AKINESIA DEFORMATION SEQUENCE
  GRANULOMATOUS DISEASE, CHRONIC, AUTOSOMAL RECESSIVE, CYTOCHROME B-POSITIVE,
  INTESTINAL ATRESIA, MULTIPLE
  LEBER CONGENITAL AMAUROSIS 4
  MYASTHENIC SYNDROME, CONGENITAL, ASSOCIATED WITH ACETYLCHOLINE RECEPTOR
  NEPHRONOPHTHISIS 12
  PEROXISOME BIOGENESIS DISORDER 2A (ZELLWEGER)
  PITUITARY ADENOMA PREDISPOSITION
  RETINITIS PIGMENTOSA 60
  TRICHOHEPATOENTERIC SYNDROME 1 (THES1)
  TYPE II


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Range on the Protein:  

   Protein ID            Protein Position

Domain Position:  


Feature Name:Total Found:
TPR motif
binding surface












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Please Cite: Peterson, T.A., Adadey, A., Santana-Cruz ,I., Sun, Y., Winder A, Kann, M.G., (2010) DMDM: Domain Mapping of Disease Mutations. Bioinformatics 26 (19), 2458-2459.

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