Background The clinical phenotyping of patients with achromatopsia harboring variants in phosphordiesterase 6C (PDE6C) has poorly been described in the literature. PDE6C encodes the catalytic subunit of the cone phosphodiesterase, which hydrolyzes the cyclic guanosine monophosphate that proceeds with the hyperpolarization of photoreceptor cell membranes, as the final step of the phototransduction cascade. Methods In the current study, two patients from a consanguineous family underwent full ophthalmologic examination and molecular investigations including WES. The impact of the variant on the functionality of the protein has been analyzed using in silico molecular modeling.  https://www.selleckchem.com/products/lys05.html  Results The patients identified with achromatopsia segregated a homozygous missense variant (c.C1775Ap.A592D) in PDE6C gene located on chromosome 10q23. Molecular modeling demonstrated that the variant would cause a protein conformational change and result in reduced phosphodiesterase activity. Conclusion Our data extended the phenotypic spectrum of retinal disorders caused by PDE6C variants and provided new clinical and genetic information on achromatopsia.Background The present study aimed to evaluate the effects of different concentrations of cerium oxide nanoparticles (CONPs) on the oxidative stress (OS) status in kidney, lung, and serum of rats. Methods Male Wistar Rats were treated intraperitoneally with 15, 30, and 60 mg/kg/day of CONPs. The biochemical parameters, including total antioxidant capacity (TAC), total thiol group (TTG), malondialdehyde (MDA), SOD (superoxide dismutase), and catalase (CAT) were assayed in serum, kidney, and lung tissues. Results MDA decreased, but TTG and CAT increased in serum by the administration of CONPs at 15 mg/kg. In kidney homogenate obtained from the group treated with CONPs at 15 mg/kg, TAC, TTG, and CAT significantly increased compared to the control group. However, CONPs at 15, 30, and 60 mg/kg significantly decreased MDA level compared to the control group. In lung tissue, CONPs in doses of 15, 30 and 60 mg/kg significantly decreased CAT activity, TTG and TAC compared to the control group, while in kidney tissue, CONPs at the concentrations of 30 and 60 mg/kg significantly increased MDA compared to the control group. Conclusion Our findings suggest that CONPs attenuate OS in the kidney and affect the serum levels of OS-related markers but induce OS in the lung tissue in a dose-dependent manner.Background Our previous findings indicated that carvacrol and thymol alleviate cognitive impairments caused by Aβ in rodent models of Alzheimer's disease (AD). In this study, the neuroprotective effects of carvacrol and thymol against Aβ25-35-induced cytotoxicity were evaluated, and the potential mechanisms were determined. Methods PC12 cells were pretreated with Aβ25-35 for 2 h, followed by incubation with carvacrol or thymol for additional 48 h. Cell viability was measured by the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide method. A flurospectrophotometer was employed to observe the intracellular reactive oxygen species (ROS) production. Protein kinase C (PKC) activity was analyzed using ELISA. Results Our results indicated that carvacrol and thymol could significantly protect PC12 cells against Aβ25-35-induced cytotoxicity. Furthermore, Aβ25-35 could induce intracellular ROS production, while carvacrol and thymol could reverse this effect. Moreover, our findings showed that carvacrol and thymol elevate PKC activity similar to Bryostatin-1, as a PKC activator. Conclusion This study provided the evidence regarding the protective effects of carvacrol and thymol against Aβ25–35-induced cytotoxicity in PC12 cells. The results suggested that the neuroprotective effects of these compounds against Aβ25-35 might be through attenuating oxidative damage and increasing the activity of PKC as a memory-related protein. Thus, carvacrol and thymol were found to have therapeutic potential in preventing or modulating AD.Background Through combining two synthetic and natural polymers, scaffolds can be developed for tissue engineering and regenerative medicine purposes. Methods In this work, carboxymethyl chitosan (CMC; 20%) was grafted to Polycaprolactone (PCL) nanofibers using the cold atmospheric plasma of helium. The PCL scaffolds were exposed to CAP, and functional groups were developed on the PCL surface. Results The results of Fourier Transform Infrared Spectroscopy confirmed CMC (20%) graft on PCL scaffold. The Thiazolyl blue tetrazolium bromide assay showed a significant enhancement (p less then 0.05) in the cell affinity and proliferation of adipose-derived stem cells (ADSCs) to CMC20%-graft-PCL scaffolds. After 14 days, bone differentiation was affirmed through alizarin red and calcium depositions. Conclusion Based on the results, the CMC20%-graft-PCL can support the proliferation of ADSCs and induce the differentiation into bone with longer culture time.Background The most important cause of neurodegeneration in Alzheimer's disease (AD) is associated with inflammation and oxidative stress. Probiotics are microorganisms that are believed to be beneficial to human and animals. Probiotics reduce oxidative stress and inflammation in some cases. Therefore, this study determined the effects of probiotics mixture on the biomarkers of oxidative stress and inflammation in an AD model of rats. Methods In this study, 50 rats were allocated to five groups, namely control, sham, and AD groups with Aβ1-40 intra-hippocampal injection, as well as AD + rivastigmine and AD + probiotics groups with Aβ1-40 intra-hippocampal injection and 2 ml (1010 CFU) of probiotics (Lactobacillus reuteri, Lactobacillus rhamnosus, and Bifidobacterium infantis) orally once a day for 10 weeks. MWM was used to assess memory and learning. To detect Aβ plaque, Congo red staining was used. Oxidative stress was monitored by measuring the MDA level and SOD activity, and to assess inflammation markers (IL-1β and TNF-α) in the hippocampus, ELISA method was employed.. Results Spatial memory improved significantly in treatment group as measured by MWM. Probiotics administration reduced Aβ plaques in AD rats. MDA decreased and SOD increased in the treatment group. Besides, probiotics reduced IL-1β and TNF-α as inflammation markers in the AD model of rats. Conclusion Our data revealed that probiotics are helpful in attenuating inflammation and oxidative stress in AD.