Expansion over 200 CGG repeats in FMR1 gene causes inherited intellectual disability or autism spectrum disorder named as fragile X syndrome. Despite the known cause fragile X syndrome pathogenesis has not been specified yet. The ICGi026-A iPSCs line was obtained by the reprogramming of the peripheral blood mononuclear cells from a 9-year-old boy with fragile X syndrome. The ICGi026-A iPSCs expressed pluripotency markers, had a normal male karyotype (46, XY) and had the capacity to in vivo differentiate into the cells of three germ layers.Severe mycological epilepsy of infancy is a catastrophic disease with preferential dysfunction of interneurons, frequentepisoderate, cognitive and sudden death. The disease is mainly caused by heterozygous loss-of-function mutation of SCN1A gene encoding α subunit of the sodium channel Nav1.1. To generate mutations in normal iPSC, Transcription activator-like effector nucleases was used to introduce the epilepsy-causing mutation A5768G into the endogenous locus of SCN1A gene. The gene editing induced pluripotent stem cell line and normal iPSC were obtained from the same donor to eliminate significantly the genetic background noise.Poly-β-hydroxybutyrate (PHB) is a biodegradable biopolymer that may replace fossil-based plastics reducing its negative environmental impact. One highly sustainable strategy to produce these biopolymers is the exploitation of photosynthetic microorganisms that use sunlight and CO2 to produce biomass and subsequently, PHB. Exploring environmental biological diversity is a powerful tool to find resilient microorganisms potentially exploitable to produce bioproducts. In this work, a cyanobacterium (Synechocystis sp.) isolated from a contaminated area close to an important industrial complex was shown to produce PHB under different culture conditions. Carbon, nutrients supply and light intensity impact on biomass and PHB productivity were assessed, showing that the highest yield of PHB achieved was 241 mg L-1 (31%dcw) under high light intensity. Remarkably this condition not only stimulated PHB accumulation by 70% compared to other conditions tested but also high cellular duplication rate, maximizing the potential of this strain for PHB production.Internal resistance is one of the limiting factors for power production in microbial fuel cells (MFC). To overcome this, current study designed polyaniline functionalized activated carbon (PANi-FAC) composite as capacitive anode with strategic electrocatalytic capability, and was comparatively assessed with SSM-PANi and bare SSM as anodes in three double chambered MFCs respectively. Power output and COD removal efficiency of PANi-FAC coated on stainless steel mesh (SSM-PANi/FAC) is superior (322 mW/m2; 87.6%) in comparison to SSM-PANi (273 mW/m2; 62.4%) and bare SSM (169 mW/m2; 54%). In addition, maximum specific capacitance of hybrid electrodes is relatively high with SSM-PANi/FAC (360.84 F/g) than SSM-PANi anode (128.26 F/g). Nyquist impedance plots showed less charge-transfer resistance (Rct) with SSM-PANi/FAC (29.9 Ω) than SSM-PANi (206.8 Ω) and SSM anodes (678 Ω). Study infers that, development of electrochemical double layer capacitance makes SSM-PANi/FAC, a potential capacitive anode for augmenting bio-electrocatalytic activity and reducing Ohmic losses.This study investigates the influence of pH on protein conversion into volatile fatty acids by anaerobic mixed-culture fermentation, a topic that, in contrast to glucose fermentation, only had scarce and contradictory information available. Several experiments were performed with two model proteins (casein and gelatin) at three different pH values (5, 7 and 9) using chemostats and batch tests.  https://www.selleckchem.com/products/pfi-2.html  Highest conversion was reached at neutral pH although complete acidification was never achieved. Longer chain carboxylates production was favoured at low pH, while acetic acid was the main product at pH 7 and 9. Amino acids preferential consumption also varied with pH and protein composition. In fact, protein conversion stoichiometry is mainly driven by energetic yields and amino acid molecular configuration. Overall, this study identifies pH adjustment as a way to steer volatile fatty acid production during mixed-culture fermentation of proteins.
  It remains challenging to make a differential diagnosis between atypical parkinsonism and Parkinson's disease (PD) from routine neuroimaging. This case-control study aimed to quantitatively investigate both morphological and signal intensity changes in susceptibility weighted imaging (SWI) of the lentiform nucleus (LN) for discriminating parkinsonism-predominant multiple system atrophy (MSA-P) from PD.
 
  We retrospectively enrolled patients with MSA-P, PD, and sex- and age-matched controls between January 2016 and November 2019at the Movement Disorder Center who underwent 3T MR imaging of brain with SWI sequence. Two specialists at the center reviewed the medical records and made the final diagnosis, and two experienced neuroradiologists performed MRI image analysis based on a defined radiological protocol to conduct the ROI-based morphological measurements of the LN and the signal intensity.
 
  A total of 19 patients with MSA-P, 19 patients with PD and 19 controls were enrolled in this study. We found that patients with MSA-P had significant decreases size in the short line (SL) and the ratio of the SL and the long line (SLLr) and increased value in the signal intensity standard deviation of the LN (SIsd_LN) compared with the patients with PD and with the controls (P<0.05). Combining these three indexes, this finding had a sensitivity of 94.7% and a specificity of 63.2% to distinguish MSA-P from PD.
 
  As compared to PD and control subjects, the SA-P patients are characterized by narrowing morphology and the inhomogeneous signal intensity of the posterior region of LN. The quantitative morphological change is a possible potential marker to differentiate MSA-P from PD on SWI.
 As compared to PD and control subjects, the SA-P patients are characterized by narrowing morphology and the inhomogeneous signal intensity of the posterior region of LN. The quantitative morphological change is a possible potential marker to differentiate MSA-P from PD on SWI.