Objective To analyze the clinical features and NDP gene variants in patients with Norrie disease and familial exudative vitreoretinopathy,(FEVR).Methods Sixteen patients who were diagnosed as FEVR and 3 patients who were diagnosed as Norrie disease underwent detailed ocular examinations in Beijing Tongren Hospital from 2012 to 2018.Peripheral venous blood was drawn from patients and their family members for extration of genomic DNA.All exons of NDP gene were analyzed by direct sequencing of PCR-amplified DNA fragments.This study was approved by Joint Committee on Clinical Investigation of Beijing Tongren Hospital,and patients information and venous blood collection were done after the informed consents were obtained.Results Four unreported NDP gene mutations were detected in one FEVR patient and 3 Norrie disease patients:c.217T＞C (p.S73P),c.2T＞C (p.M1),c.194G＞T (p.C65F) and c.384C＞G (p.C128W).One patient who was clinically diagnosed as FEVR had no symptom but the avascular zone on the bilateral peripheral retina.The three Norrie disease patients who were clinically diagnosed as Norrie disease all appearred to have bilateral congenital blindness,mass in the vitreous and retinal detachment,and one in the three Norrie disease patient had evident mental retardation.Conclusions The results of this paper extend NDP gene mutation spectrum,and NDP gene is further confirmed to be the casuse of Norrie disease.

OBJECTIVES: Public service motivation refers to the idea of commitment to the public service, pursuit of the public interest, and the desire to perform work that is worthwhile to society. This study investigates how challenge stress and hindrance stress influence job performance among healthcare workers in Chinese public hospitals. It has also examined the mediating effect of public service motivation. METHODS: Data of 1594 healthcare workers were obtained from typical public hospitals in eastern, central, and western China. To test our hypotheses, we used descriptive statistical analysis, correlation analysis, structural equation modeling, and subgroup analysis to investigate the sample. RESULTS: Challenge stress and hindrance stress were strongly correlated among healthcare workers in Chinese public hospitals (β = 0.59; p < 0.001). Challenge stress was significantly positively associated with public service motivation (β = 0.14; p < 0.001) and job performance (β = 0.13; p < 0.001). Hindrance stress was significantly negatively associated with public service motivation (β = - 0.27; p < 0.001) and job performance (β = - 0.08; p < 0.05). Public service motivation was directly positively associated with job performance (β = 0.58; p < 0.001), and it indirectly mediated the association between job stress and job performance. CONCLUSIONS This study provides important empirical evidence on the effects of job stress and public service motivation on job performance among healthcare workers in Chinese public hospitals. Job performance may be raised by limiting hindrance stress, which provides moderate challenge stress and increases public service motivation.
Adult ; China ; Cross-Sectional Studies ; Female ; Health Personnel ; psychology ; Hospitals, Public ; Humans ; Male ; Middle Aged ; Motivation ; Occupational Stress ; psychology ; Public Sector ; Regression Analysis ; Self Report ; Work Performance ; Workplace ; psychology ; Young Adult

Mannosylerythritol lipids (MELs), mainly produced by Ustilago and Pseudozyma, are surface active compounds that belong to the glycolipid class of biosurfactants. MELs have potential application in food, pharmaceutical and cosmetics industries due to their excellent surface activities and other peculiar bioactivities. In recent years, the research field of MELs has regained much attention abroad. However, MELs are rarely studied in China. In this review, the producing microorganisms and production conditions, diverse structures, biochemical properties, structure-function relationship and biosynthetic pathways of MELs are described. Some research problems and prospects are summarized and discussed as well.
Glycolipids ; biosynthesis ; genetics ; Metabolic Networks and Pathways ; genetics ; Surface-Active Agents ; metabolism ; Ustilaginales ; classification ; genetics ; metabolism ; Ustilago ; genetics ; metabolism