Harmonin

spinqualitylabelsall models Human harmonin N-terminal and PDZ1 domain (skyblue) complex with Usher syndrome type 1G protein (green) (PDB code 3k1r) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Function Harmonin (Harm) or Usher syndrome protein is a scaffold PDZ-containing protein which integrates three Usher syndrome (USH) type proteins to USH1-protein network. Harm and cadherin 23 form a complex by PDZ domain interactions. Disease A defect in harmonin which is expressed in the inner ear hair cells causes USH type 1C. Defects in the formation of USH-protein complex are predicted to disrupt stereocilia bundles and cause deafness in USH1 patients. Structural highlights Harm R103 forms a salt bridge with D458 of Usher syndrome type 1G protein and S115 forms hydrogen bond with it. The mutation of D458V in the Usher syndrome type 1G protein causes deaf-blindness.

3D Structures of harmonin

Updated on 14-May-2019

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• Harmonin N-terminal
  • 2kbq – hHarm N-terminal – human - NMR
    
  • 2lsr, 2kbr – hHarm N-terminal + cadherin peptide - NMR
    
• Harmonin N-terminal + PDZ1 domain residues 1-192
  • 3k1r – hHarm N-terminal + PDZ1 domain + Usher syndrome type-1g protein SAM-PBM domain
    
  • 5f3x – hHarm N-terminal + PDZ1 domain + ankyrin SAM-PBM domain
    
• Harmonin PDZ2 domain residues 201-301
  • 1x5n – hHarm PDZ2 domain - NMR
    
  • 2kbs – hHarm PDZ2 domain + cadherin peptide - NMR
    
• Harmonin PDZ3 domain residues 380-484
  • 5mv8, 5mv9, 5xbf – hHarm PDZ3 domain residues 380-484 + unconventional myosin
    
• Harmonin PDZ4 domain residues 438-548
  • 1v6b – Harm PDZ4 domain residues 438-548 – mouse - NMR
    

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