Fibrodysplasia ossificans progressiva, an ultra-rare and disabling genetic disorder of skeletal malformations and progressive heterotopic ossification (HO), is the most catastrophic condition of skeletal metamorphosis in humans. We studied 72 patients with FOP in China and analyzed their phenotypes and genotypes comprising the world’s largest ethnically homogeneous population of FOP patients. Ninety-nine percent of patients (71/72 cases) were of Han nationality; one percent of patients (1/72 cases) were of Hui nationality. Based on clinical examination, ninety-two percent of patients (66/72 cases) had classic FOP; four percent of patients (3/72 cases) were FOP-plus, and four percent of patients (3/72) were FOP variants. Importantly, all individuals with FOP had mutations in the protein-coding region of Activin A Receptor, Type I/Activin-like kinase 2 (ACVR1/ALK2). Ninety-seven percent of FOP patients (70/72 cases) had the canonical c.617G>A (p.R206H) mutation, while three percent of FOP patients (2/72 cases) had variant mutations in ACVR1/ALK2. Taken together, the genotypes and phenotypes of individuals with FOP from the Han nationality in China are similar to those reported elsewhere and supports the fidelity of this ultra-rare disorder in the world’s most highly populated nation and across wide racial, ethnic, gender and geographic distributions.

We herein report the unprecedented synthesis of diverse biologically important aza-heterocycles by employing sulfilimines as nitrene transfer reagents.T his class of sulfurbased aza-ylides had not been successfully used for gold nitrene transfer before.T his work contains an efficient generation of a-imino gold carbenes by N À Sc leavage of sulfilimines.T hese gold carbenes undergo C À Hi nsertion, cyclopropanation, and nucleophilic attacktoform indoles (44 examples), 3-azabicyclo[3.1.0]hexan-2-imines (24 examples), and imidazoles (3 examples).Our study represents au nique gold-catalyzed reaction between alkynes and sulfur ylides,and also includes the first aza-heterocycle synthesis that proceeds by intermolecular nitrene transfer followed by cyclopropanation of the a-imino gold carbenes.M oreover,a nu nexpected synthesis of 4-acylquinolines (3 examples) from 2-acylphenyl sulfilimines and propargylic silyl ether derivatives by a1 ,2hydride shift onto the a-imino gold carbene and asubsequent Mukaiyama aldol cyclization was discovered.Supportinginformation and the ORCID identification number(s) for the author(s) of this article can be found under: https://doi.

Catalytic approaches to pharmaceutically important bioactive skeletons through gold carbene intermediates have experienced a dramatic development in the last decade. Although various carbene precursors continue to play an important role in heterocyclic syntheses, these reagents are associated with some drawbacks in terms of functional group tolerance, synthetic methods and safety limitations. A new generation of nitrene transfer reagents was established in 2019: the sulfilimines. These are safe, inexpensive and readily available. They can conveniently be stored and handled, and thus represent ideal reagents for the fast and modular modification of scaffolds and the preparation of libraries by intermolecular reactions of two components. Both the practical methods for synthesizing sulfilimines and the versatility of these ylidic species in gold‐catalyzed preparation of structural diversity, for both heterocycles and carbocycles, will be outlined in this Concept article.

Solar-driven interfacial evaporation has been considered as a potential pathway for yielding freshwater from seawater. However, the significant reduction of the evaporation rate has become an immediate problem because of the inevitable salt accumulation on evaporators during long-term evaporation, especially for highly saline water. Here, a novel efficient linen fiber-based solar steam generator by combination of broadband light absorption, superhydrophilicity, and a unique transition layer of water between a solar evaporator and a thermal insulator for simultaneously enhanced thermal management and water supply is reported. Through coupling of enhanced thermal management and sufficient water supply, the as-prepared solar steam generator achieves an excellent evaporation rate of 1.447 kg•m −2 •h −1 and a conversion efficiency of over 90% under 1 sun irradiation in 20 wt % saline water. Our investigation in this work may open up a simple and new avenue to design and fabricate a low cost and effective solar receiver toward highly saline water desalination.