PURPOSE Axenfeld-Rieger (AR) is an autosomal dominant disorder with phenotypic heterogeneity characterized by anterior segment dysgenesis, facial bone defects, and redundant periumbilical skin. The PITX2 gene, on chromosome 4q25, and the FOXC1 gene, on chromosome 6p25, have been implicated in the different phenotypes of the syndrome through mutational events. Recently, the CYP1B1 gene was found...

RGS proteins are GTPase activators for heterotrimeric G proteins. We report here the 2.8 A resolution crystal structure of the RGS protein RGS4 complexed with G(i alpha1)-Mg2+-GDP-AlF4 . Only the core domain of RGS4 is visible in the crystal. The core domain binds to the three switch regions of G(i alpha1), but does not contribute catalytic residues that directly interact with either GDP or AlF...

Regulators of G-protein signalling (RGS proteins) are a family of highly diverse, multifunctional signalling proteins that share a conserved 120 amino acid domain (RGS domain). RGS domains bind directly to activated Galpha subunits and act as GTPase-activating proteins (GAPs) to attenuate and/or modulate hormone and neurotransmitter receptor-initiated signalling by both Galpha-GTP and Gbetagamm...

The second author’s name is spelled incorrectly. The correct name is: Lauren A. Howell. The correct citation is: Korrapati MC, Howell LA, Shaner BE, Megyesi JK, Siskind LJ, et al. (2013) Suramin: A Potential Therapy for Diabetic Nephropathy. PLoS ONE 8(9): e73655. doi:10.1371/journal.pone.0073655 The Author Contributions should read: Conceived and designed the experiments: MCK LJS RGS. Performe...

Heterotrimeric G proteins of the G12 family regulate the Rho GTPase through RhoGEFs that contain an amino-terminal regulator of G protein signaling (RGS) domain (RGS-RhoGEFs). Direct regulation of the activity of RGS-RhoGEFs p115 or leukemia-associated RhoGEF (LARG) by Galpha13 has previously been demonstrated. However, the precise biochemical mechanism by which Galpha13 stimulates the RhoGEF a...

Administration of a tumour-seeking compound labeled with a low-energy isotope and intraoperative screening with the gamma probe (radioguided surgery, RGS) could be useful in reoperations for advanced neuroblastoma when the normal anatomy is altered. A pilot study was performed to test the feasibility of this technique. Five patients underwent six relaparotomies for recurrent stage III or IV neu...

Aims. By measuring the gravitational redshift of spectral features in the spectrum of thermal radiation emitted from neutron stars, useful constraints for the equation of state of superdense matter can be obtained via an estimate of the mass-to-radius ratio. We searched for spectral line features in the high-resolution X-ray spectrum of the isolated neutron star RX J0720.4−3125. Methods. Our ta...

We report the spectral analysis of a long XMM-Newton observation of the well-studied, moderate luminosity Broad Line Seyfert 1 galaxy NGC 5548. The source was at an historically average brightness and we find the hard (3-10 keV) spectrum can be well fitted by a power law of photon index Γ∼1.75, together with reflection. The only feature in the hard X-ray spectrum is a narrow emission line near ...

Regulator of G protein signaling (RGS) proteins accelerate the endogenous GTPase activity of Galpha(i/o) proteins to increase the rate of deactivation of active Galpha-GTP and Gbetagamma signaling molecules. Previous studies have suggested that RGS proteins are more effective on less efficiently coupled systems such as with partial agonist responses. To determine the role of endogenous RGS prot...

Patients affected by vestibular schwannomas typically report a number of symptoms and minor disabilities after surgery. Therefore, surgeons dealing with this pathology should also try to achieve a good QoL for patients who have undergone tumour removal. The aim of this study was to analyse QoL in subjects undergoing surgery for vestibular schwannomas and to try to establish a relationship with ...