Giant Kohn anomaly and the phase transition in charge density wave ZrTe_3

A strong Kohn anomaly in ZrTe_3 is identified in the mostly transverse acoustic phonon branch along the modulation vector q_P with polarization along the a* direction. This soft mode freezes to zero frequency at the transition temperature T_P and the temperature dependence of the frequency is strongly affected by fluctuation effects. Diffuse x-ray scattering of the incommensurate superstructure shows a power law scaling of the intensity and the correlation length that is compatible with an order parameter of dimension n = 2.Comment: 4 pages, 4 figures. accepted at Phys. Rev. Let

Animal models for pah and increased pulmonary blood flow

Pulmonary arterial hypertension (PAH), a progressive pulmonary vasoproliferative disorder, is characterized by the development of unique neointimal lesions including concentric laminar intimal fibrosis and plexiform lesions.In PAH associated with congenital heart disease, increased pulmonary blood flow (i.e., systemic-to-pulmonary shunt) is an essential trigger for the occurrence of neointimal lesions and disease development. Although neointimal development is well described histopathologically, the pathogenesis of flow-induced PAH and its typical vascular lesions is largely unknown.Animal models play a crucial part in giving insight in new pathobiological processes in PAH and possible new therapeutic targets. However, as for any preclinical model, the pathophysiological mechanism and clinical course have to be comparable to the human disease that it is supposed to mimic. This means that animal models mimicking human PAH ideally are characterized by (1) a hit resembling the human disease, (2) specific vascular remodeling that resembles neointimal development in human PAH, and (3) progressive disease development that leads to right ventricular (RV) dysfunction and eventually death.Therefore, this chapter will discuss currently used animal models for pulmonary hypertension that are of interest for PAH in the pediatric population, specifically PAH associated with congenital heart disease. Since increased pulmonary blood flow is known to be a trigger for PAH development in this population, particular emphasis will be put on models with increased pulmonary blood flow.</p

Animal models for pah and increased pulmonary blood flow

Pulmonary arterial hypertension (PAH), a progressive pulmonary vasoproliferative disorder, is characterized by the development of unique neointimal lesions including concentric laminar intimal fibrosis and plexiform lesions.In PAH associated with congenital heart disease, increased pulmonary blood flow (i.e., systemic-to-pulmonary shunt) is an essential trigger for the occurrence of neointimal lesions and disease development. Although neointimal development is well described histopathologically, the pathogenesis of flow-induced PAH and its typical vascular lesions is largely unknown.Animal models play a crucial part in giving insight in new pathobiological processes in PAH and possible new therapeutic targets. However, as for any preclinical model, the pathophysiological mechanism and clinical course have to be comparable to the human disease that it is supposed to mimic. This means that animal models mimicking human PAH ideally are characterized by (1) a hit resembling the human disease, (2) specific vascular remodeling that resembles neointimal development in human PAH, and (3) progressive disease development that leads to right ventricular (RV) dysfunction and eventually death.Therefore, this chapter will discuss currently used animal models for pulmonary hypertension that are of interest for PAH in the pediatric population, specifically PAH associated with congenital heart disease. Since increased pulmonary blood flow is known to be a trigger for PAH development in this population, particular emphasis will be put on models with increased pulmonary blood flow.</p

Animal models for pah and increased pulmonary blood flow

Pulmonary arterial hypertension (PAH), a progressive pulmonary vasoproliferative disorder, is characterized by the development of unique neointimal lesions including concentric laminar intimal fibrosis and plexiform lesions.In PAH associated with congenital heart disease, increased pulmonary blood flow (i.e., systemic-to-pulmonary shunt) is an essential trigger for the occurrence of neointimal lesions and disease development. Although neointimal development is well described histopathologically, the pathogenesis of flow-induced PAH and its typical vascular lesions is largely unknown.Animal models play a crucial part in giving insight in new pathobiological processes in PAH and possible new therapeutic targets. However, as for any preclinical model, the pathophysiological mechanism and clinical course have to be comparable to the human disease that it is supposed to mimic. This means that animal models mimicking human PAH ideally are characterized by (1) a hit resembling the human disease, (2) specific vascular remodeling that resembles neointimal development in human PAH, and (3) progressive disease development that leads to right ventricular (RV) dysfunction and eventually death.Therefore, this chapter will discuss currently used animal models for pulmonary hypertension that are of interest for PAH in the pediatric population, specifically PAH associated with congenital heart disease. Since increased pulmonary blood flow is known to be a trigger for PAH development in this population, particular emphasis will be put on models with increased pulmonary blood flow.</p

Animal models for pah and increased pulmonary blood flow

Pulmonary arterial hypertension (PAH), a progressive pulmonary vasoproliferative disorder, is characterized by the development of unique neointimal lesions including concentric laminar intimal fibrosis and plexiform lesions.In PAH associated with congenital heart disease, increased pulmonary blood flow (i.e., systemic-to-pulmonary shunt) is an essential trigger for the occurrence of neointimal lesions and disease development. Although neointimal development is well described histopathologically, the pathogenesis of flow-induced PAH and its typical vascular lesions is largely unknown.Animal models play a crucial part in giving insight in new pathobiological processes in PAH and possible new therapeutic targets. However, as for any preclinical model, the pathophysiological mechanism and clinical course have to be comparable to the human disease that it is supposed to mimic. This means that animal models mimicking human PAH ideally are characterized by (1) a hit resembling the human disease, (2) specific vascular remodeling that resembles neointimal development in human PAH, and (3) progressive disease development that leads to right ventricular (RV) dysfunction and eventually death.Therefore, this chapter will discuss currently used animal models for pulmonary hypertension that are of interest for PAH in the pediatric population, specifically PAH associated with congenital heart disease. Since increased pulmonary blood flow is known to be a trigger for PAH development in this population, particular emphasis will be put on models with increased pulmonary blood flow.</p

Animal models for pah and increased pulmonary blood flow

Pulmonary arterial hypertension (PAH), a progressive pulmonary vasoproliferative disorder, is characterized by the development of unique neointimal lesions including concentric laminar intimal fibrosis and plexiform lesions.In PAH associated with congenital heart disease, increased pulmonary blood flow (i.e., systemic-to-pulmonary shunt) is an essential trigger for the occurrence of neointimal lesions and disease development. Although neointimal development is well described histopathologically, the pathogenesis of flow-induced PAH and its typical vascular lesions is largely unknown.Animal models play a crucial part in giving insight in new pathobiological processes in PAH and possible new therapeutic targets. However, as for any preclinical model, the pathophysiological mechanism and clinical course have to be comparable to the human disease that it is supposed to mimic. This means that animal models mimicking human PAH ideally are characterized by (1) a hit resembling the human disease, (2) specific vascular remodeling that resembles neointimal development in human PAH, and (3) progressive disease development that leads to right ventricular (RV) dysfunction and eventually death.Therefore, this chapter will discuss currently used animal models for pulmonary hypertension that are of interest for PAH in the pediatric population, specifically PAH associated with congenital heart disease. Since increased pulmonary blood flow is known to be a trigger for PAH development in this population, particular emphasis will be put on models with increased pulmonary blood flow.</p

Animal models for pah and increased pulmonary blood flow

Pulmonary arterial hypertension (PAH), a progressive pulmonary vasoproliferative disorder, is characterized by the development of unique neointimal lesions including concentric laminar intimal fibrosis and plexiform lesions.In PAH associated with congenital heart disease, increased pulmonary blood flow (i.e., systemic-to-pulmonary shunt) is an essential trigger for the occurrence of neointimal lesions and disease development. Although neointimal development is well described histopathologically, the pathogenesis of flow-induced PAH and its typical vascular lesions is largely unknown.Animal models play a crucial part in giving insight in new pathobiological processes in PAH and possible new therapeutic targets. However, as for any preclinical model, the pathophysiological mechanism and clinical course have to be comparable to the human disease that it is supposed to mimic. This means that animal models mimicking human PAH ideally are characterized by (1) a hit resembling the human disease, (2) specific vascular remodeling that resembles neointimal development in human PAH, and (3) progressive disease development that leads to right ventricular (RV) dysfunction and eventually death.Therefore, this chapter will discuss currently used animal models for pulmonary hypertension that are of interest for PAH in the pediatric population, specifically PAH associated with congenital heart disease. Since increased pulmonary blood flow is known to be a trigger for PAH development in this population, particular emphasis will be put on models with increased pulmonary blood flow.</p

Vastus medialis motor unit properties in knee osteoarthritis

<p>Abstract</p> <p>Background</p> <p>Maximal isometric quadriceps strength deficits have been widely reported in studies of knee osteoarthritis (OA), however little is known about the effect of osteoarthritis knee pain on submaximal quadriceps neuromuscular function. The purpose of this study was to measure vastus medialis motor unit (MU) properties in participants with knee OA, during submaximal isometric contractions.</p> <p>Methods</p> <p>Vastus medialis motor unit potential (MUP) parameters were assessed in 8 patients with knee OA and 8 healthy, sex and age-matched controls during submaximal isometric contractions (20% of maximum isometric torque). Unpaired t-tests were used to compare groups for demographic and muscle parameters.</p> <p>Results</p> <p>Maximum knee extension torque was ~22% lower in the OA group, a difference that was not statistically significantly (p = 0.11). During submaximal contractions, size related parameters of the needle MUPs (e.g. negative peak duration and amplitude-to-area ratio) were greater in the OA group (p < 0.05), with a rightward shift in the frequency distribution of surface MUP negative peak amplitude. MUP firing rates were significantly lower in the OA group (p < 0.05).</p> <p>Conclusions</p> <p>Changes in MU recruitment and rate coding strategies in OA may reflect a chronic reinnervation process or a compensatory strategy in the presence of chronic knee pain associated with OA.</p

A novel familial mutation in the PCSK1 gene that alters the oxyanion hole residue of proprotein convertase 1/3 and impairs its enzymatic activity.

Four siblings presented with congenital diarrhea and various endocrinopathies. Exome sequencing and homozygosity mapping identified five regions, comprising 337 protein-coding genes that were shared by three affected siblings. Exome sequencing identified a novel homozygous N309K mutation in the proprotein convertase subtilisin/kexin type 1 (PCSK1) gene, encoding the neuroendocrine convertase 1 precursor (PC1/3) which was recently reported as a cause of Congenital Diarrhea Disorder (CDD). The PCSK1 mutation affected the oxyanion hole transition state-stabilizing amino acid within the active site, which is critical for appropriate proprotein maturation and enzyme activity. Unexpectedly, the N309K mutant protein exhibited normal, though slowed, prodomain removal and was secreted from both HEK293 and Neuro2A cells. However, the secreted enzyme showed no catalytic activity, and was not processed into the 66 kDa form. We conclude that the N309K enzyme is able to cleave its own propeptide but is catalytically inert against in trans substrates, and that this variant accounts for the enteric and systemic endocrinopathies seen in this large consanguineous kindred