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Hyaline fibromatosis syndrome (HFS), resulting from ANTXR2 mutations, is an ultra-rare disease that causes intestinal lymphangiectasia and protein-losing enteropathy (PLE). The mechanisms leading to the gastrointestinal phenotype in these patients are not well defined. We present two patients with congenital diarrhea, severe PLE and unique clinical features resulting from deleterious ANTXR2 mutations. Intestinal organoids were generated from one of the patients, along with CRISPR-Cas9 ANTXR2 knockout, and compared with organoids from two healthy controls. The ANTXR2-deficient organoids displayed normal growth and polarity, compared to controls. Using an anthrax-toxin assay we showed that the c.155C>T mutation causes loss-of-function of ANTXR2 protein. An intrinsic defect of monolayer formation in patient-derived or ANTXR2KO organoids was not apparent, suggesting normal epithelial function. However, electron microscopy and second harmonic generation imaging showed abnormal collagen deposition in duodenal samples of these patients. Specifically, collagen VI, which is known to bind ANTXR2, was highly expressed in the duodenum of these patients. In conclusion, despite resistance to anthrax-toxin, epithelial cell function, and specifically monolayer formation, is intact in patients with HFS. Nevertheless, loss of ANTXR2-mediated signaling leads to collagen VI accumulation in the duodenum and abnormal extracellular matrix composition, which likely plays a role in development of PLE.The use of technology that uses organisms to synthesize metal nanoparticles is necessary to maintain a sustainable society. In this study, we investigated and screened the microorganisms isolated from environmental water by quantifying the reproducibility of synthetic Au nanoparticles and the ability of large amount synthesis. The microorganism (K-142) of the Bacillus genus showed the best activity in the investigation. K-142 can also synthesize Ag, CdS and PbS nanoparticles, and the deposition efficiency of Ag, Al, Cd, Cu, and Pb was about 64.8-99.2%. According to the observation results under the microscope after fluorescent staining, K-142 could survive after being treated with 0.5 mM metal solution for 24 h. Therefore, it is expected that K-142, which is easy to cultivate, would also have a high ability to reduce and deposit metal substances. K-142 can be applied to the concentration and recovery of heavy metals in environmental water, thereby opening up channels for biological water purification.Rubus coreanus Miquel (R. coreanus) is a unripen fruit of black raspberry native to eastern Asia. It is used as traditional oriental medicine and supplementary foods for centuries. Previous studies have shown that the R. coreanus extract (RCE) and its main constitute ellagic acid possess diverse biological activities. However, the effects of RCE on antitumor immunity and T cell function were not fully understood. The present study describes the anti-tumor effect of RCE in humanized PD-1 mice by blocking PD-1/PD-L1 interaction. Competitive enzyme-linked immunosorbent assay (ELISA) and pull down assay were performed to elucidate the binding properties of RCE in vitro. Cellular PD-1/PD-L1 blockade activities were measured by T cell receptor (TCR)-induced nuclear factor of activated T cells-luciferase activity in co-cultured cell models with PD-1/NFAT Jurkat and PD-L1/aAPC CHO-K1 cells. The in vivo efficacy of RCE was confirmed in humanized PD-1 mice bearing MC38 colorectal tumor. RCE and ellagic acid dose-dependently block the binding of PD-1 to PD-L1. Moreover, oral administration of RCE showed the potent anti-tumor activity similar to anti-PD-1 antibody. The present study suggests that RCE possesses potent anti-tumor effect via PD-1/PD-L1 blockade, and ellagic acid is the main compound in RCE. Thus, we provide new aspects of RCE as an immunotherapeutic agent.This paper examines the effect of the type of the emulsifying protein (EP) (sodium caseinate (SC) and whey protein isolate (WPI)) on both oil-in-water liquid-like emulsions (Es) and the corresponding cold gelled emulsions (GEs), and also the effect of addition of carob extract rich in condensed tannins (T). Bay 11-7085 manufacturer The systems, intended as functional food ingredients, were studied in various different respects, including rheological behaviour, in vitro gastrointestinal digestion with determination of the release of non-extractable proanthocyanidins (NEPA) from T, antioxidant activity and lipolysis. EP significantly affects the rheological behaviour of both Es and GEs. T incorporation produced a structural reinforcement of GEs, especially in the case of SC. The digests from Es displayed a higher antioxidant activity than those from GEs. T lipase inhibition was observed only in the formulations with WPI. Our results highlight the importance, in the design of functional foods, of analyzing different variables when incorporating a bioactive compound into a food or emulsion in order to select the better combination for the desired objective, owing to the complex interplay of the various components.There is a lack of research on Korean prospective elderly persons. In particular, there is little research regarding whether social support has a mediating effect on the relationship between physical activity and aging anxiety. Accordingly, this study investigated how social support affected physical activity and aging anxiety in 778 prospective senior citizens (55 to 65 years old) out of a total of 1447 senior citizens who participated in the Embrain Panel Power and Panel Marketing Interactive. Participants completed the IPAQ (International Physical Activity Questionnaires), Social Support Scale, and Aging Anxiety Scale. Physical activity in these Korean pre-older adults affected aging anxiety (p less then 0.001), with a fixed effect of physical activity on social support (p less then 0.001). Further, social support affected aging anxiety (p less then 0.001). Social support was also an important parameter in the relationship between physical activity and aging anxiety. In conclusion, high physical activity of pre-older Korean persons lowered their anxiety regarding aging. Social support acted as a mediator that lowered anxiety regarding aging in the most active pre-older persons.