3PVL: Structure Of Myosin Viia Myth4-Ferm-Sh3 In Complex With The Cen1 Of Sans

The unconventional myosin VIIa (MYO7A) is one of the five proteins that form a network of complexes involved in formation of stereocilia. Defects in these proteins cause syndromic deaf-blindness in humans [Usher syndrome I (USH1)]. Many disease-causing mutations occur in myosin tail homology 4-protein 4.1, ezrin, radixin, moesin (MyTH4-FERM) domains in the myosin tail that binds to another USH1 protein, Sans. We report the crystal structure of MYO7A MyTH4-FERM domains in complex with the central domain (CEN) of Sans at 2.8 angstrom resolution. The MyTH4 and FERM domains form an integral structural and functional supramodule binding to two highly conserved segments (CEN1 and 2) of Sans. The MyTH4-FERM/CEN complex structure provides mechanistic explanations for known deafness-causing mutations in MYO7A MyTH4-FERM. The structure will also facilitate mechanistic and functional studies of MyTH4-FERM domains in other myosins.
PDB ID: 3PVLDownload
MMDB ID: 88978
PDB Deposition Date: 2010/12/7
Updated in MMDB: 2011/05
Experimental Method:
x-ray diffraction
Resolution: 2.8  Å
Source Organism:
Mus musculus
Similar Structures:
Biological Unit for 3PVL: dimeric; determined by author and by software (PISA)
Molecular Components in 3PVL
Label Count Molecule
Proteins (2 molecules)
Myosin Viia Isoform 1
Molecule annotation
Usher Syndrome Type-1g Protein(Gene symbol: USH1G)
Molecule annotation
Chemicals (11 molecules)
* Click molecule labels to explore molecular sequence information.

Citing MMDB