More than 200 scientists gathered from all over the world to present the results of research spanning all areas of investigation into FRDA (including clinical aspects, FRDA pathogenesis, genetics and epigenetics of the disease, development of new models of FRDA, and drug discovery). This review provides an update on the understanding of frataxin function, developments of animal and cellular models of the disease, and recent advances in trying to uncover potential molecules for therapy.”
“Background In the transcatheter aortic valve replacement era, characterization of functional status in older adults with aortic stenosis (AS) is essential.
Gait speed (GS) is emerging as a marker of frailty and predictor of outcomes in older adults undergoing cardiovascular BKM120 in vitro intervention. The objective of this study was to delineate the prevalence of slow GS, evaluate the
association of GS with factors used in standard cardiovascular assessments, and evaluate the association of GS with dependence in activities of daily living (ADLs) in older adults with AS. Hypothesis We hypothesized that gait speed would not be associated with clinical factors, but would be associated with ADLs. Methods We evaluated GS, ADLs dependence, and Society of Thoracic Autophagy inhibitor Surgery score along with clinical and functional assessments in 102 older adults with AS being evaluated for transcatheter valve. Gait speed <0.5 m/s was considered slow, and GS NU7441 nmr =0.5 m/s was considered preserved. We assessed the association of covariates with GS as well as with ADLs dependence. Results Median GS was 0.37 m/s (interquartile range, 0.00.65 m/s). Sixty-four (63%) subjects had slow GS. Of commonly employed clinical covariates, only prior coronary intervention and serum albumin were weakly associated with GS. However, GS was independently associated with ADLs dependence (Odds ratio: 1.52 [1.21-1.91] for every 0.1 m/s decrease in GS; P = 0.0003). Conclusions Although the prevalence of slow GS in a population of elderly patients with severe AS being screened for transcatheter valve was
high, there were only weak associations between GS and other risk stratifying tools. The strong association between GS and dependent functional status suggests that assessment of gait speed is a useful, objectively measurable, risk stratification tool in this population. Dr. Maurer is supported by a grant from the NIH/NIA AG036778-02. The other authors have no funding, financial relationships, or conflicts of interest to disclose.”
“N-Cadherin and beta-catenin form a transsynaptic adhesion complex required for spine and synapse development. In adulthood, N-cadherin mediates persistent synaptic plasticity, but whether the role of N-cadherin at mature synapses is similar to that at developing synapses is unclear.