Defects in myosin VIIa, harmonin (a PDZ domain protein), cadherin 23, protocadherin 15 and sans (a putative scaffolding protein), underlie five forms of Usher syndrome type I (USH1). Mouse mutants for all these proteins exhibit disorganization of their hair bundle, which is the mechanotransduction receptive structure of the inner ear sensory cells, the cochlear and vestibular hair cells. We hav...

A recently developed integrative approach combining varied types of experimental data has been successfully applied to three-dimensional modelling of larger biomacromolecular complexes. Deuteration-assisted small-angle neutron scattering (SANS) plays a unique role in this approach by making it possible to observe selected components in the complex. It enables integrative modelling of biomolecul...

The human Usher syndrome (USH) is a complex ciliopathy with at least 12 chromosomal loci assigned to three clinical subtypes, USH1-3. The heterogeneous USH proteins are organized into protein networks. Here, we identified Magi2 (membrane-associated guanylate kinase inverted-2) as a new component of the USH protein interactome, binding to the multifunctional scaffold protein SANS (USH1G). We sho...

The hereditary hearing-vision loss disease, Usher syndrome I (USH1), is caused by defects in several proteins that can interact with each other in vitro. Defects in USH1 proteins are thought to be responsible for the developmental and functional impairments of sensory cells in the retina and inner ear. Harmonin/USH1C and Sans/USH1G are two of the USH1 proteins that interact with each other. Har...

Stochastic activity networks (SANs) are a stochastic generalization of Petri nets. The original definition of SANs has been used as a modeling formalism in several well-known and powerful modeling tools. SANs have been used to evaluate a wide range of systems, mainly for dependability and performability evaluation purposes. SAN models have not been considered in the definition of the Petri net ...

Small-angle neutron scattering (SANS) is a sensitive technique that is able to probe the structure of polymer-grafted nanoparticles and free polymer chains. Here, we combine SANS measurements with self-consistent field theory (SCFT) calculations to study the structure of deuterated poly(methyl methacrylate) (dPMMA) nanocomposites containing PMMA-grafted Fe3O4 nanospheres, with a specific emphas...

Stochastic Automata Networks (SANs) are widely used in modeling many practical systems such as queueing systems, communication systems and manufacturing systems. For the performance analysis purposes one needs to calculate the steady state distributions of SANs. Usually, the steady state distributions have no close form solutions and cannot be obtained eeciently by direct methods such as LU dec...

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