Longitudinal assessment of physical function in adults with X-linked hypophosphatemia following initiation of burosumab therapy.

Unlabelled: We assessed multiple components of muscle function in ten adults with X-linked hypophosphatemia (XLH) receiving burosumab treatment. Lower limb power (+ 9%), short physical performance battery (SPPB) score (+ 1.2 points), and physical activity (+ 65%) increased following 6 months of treatment, and hand grip increased (+ 10%) between 6 and 12 months of treatment.

Dialumene-Mediated Production of Phosphines through P Reduction.

The formation of phosphorus-rich alanes featuring butterfly-like geometries is achieved. The two-electron reduction products feature a unique P structure and can act as a source of P. The treatment of these phosphorus containing products with electrophiles under mild conditions results in the formation of different phosphines.

Reversible [4 + 1] Cycloaddition of Arenes by a "Naked" Acyclic Aluminyl Compound.

The large steric profile of the N-heterocyclic boryloxy ligand, -OB(NDippCH), and its ability to stabilize the metal-centered HOMO, are exploited in the synthesis of the first example of a "naked" acyclic aluminyl complex, [K(2.2.2-crypt)][Al{OB(NDippCH)}].

Mercury-Group 13 Metal Covalent Bonds: A Systematic Comparison of Aluminyl, Gallyl and Indyl Metallo-ligands.

Bimetallic compounds containing direct metal-group 13 element bonds have been shown to display unprecedented patterns of cooperative reactivity towards small molecules, which can be influenced by the identity of the group 13 element. In this context, we present here a systematic appraisal of group 13 metallo-ligands of the type [(NON)E] (NON=4,5-bis(2,6-diisopropylanilido)-2,7-di-tert-butyl-9,9-dimethylxanthene) for E=Al, Ga and In, through a comparison of structural and spectroscopic parameters associated with the trans L or X ligands in linear d complexes of the types LM{E(NON)} and XM'{E(NON)}. These studies are facilitated by convenient syntheses (from the In(I) precursor, InCp) of the potassium indyl species [{K(NON)In}⋅KCp] (1) and [(18-crown-6)KCp] [(NON)In] (1'), and lead to the first structural characterisation of Ag-In and Hg-E (E=Al, In) covalent bonds.

Taming Heavier Group 14 Imine Analogues: Accessing Tin Nitrogen [Sn=N] Double Bonds and their Cycloaddition/Metathesis Chemistry.

A systematic study to access stable stannaimines is reported, by combining different heteroleptic stannylenes with a range of organic azides. The reactions of terphenyl-/hypersilyl-substituted stannylenes yield the putative tin nitrogen double bond, but is directly followed by 1,2-silyl migration to give Sn systems featuring bulky silylamido ligands. By contrast, the transition from a two σ donor ligand set to a mixed σ-donor/π-donor scaffold allows access to three new stannaimines which can be handled at room temperature.

Wear resistance of an additively manufactured high-carbon martensitic stainless steel.

The dry sliding wear behaviour of a high carbon martensitic stainless steel (HCMSS) consisting of ~ 22.5 vol% of chromium (Cr)- and vanadium (V)-rich carbides processed by electron beam melting (EBM) has been captured. The microstructure consisted of martensite and retained austenite phases with a homogeneous distribution of sub-micron-sized V-rich and micron-sized Cr-rich carbides, leading to relatively high hardness.

Coordination and Homologation of CO at Al(I): Mechanism and Chain Growth, Branching, Isomerization, and Reduction.

Homologation of carbon monoxide is central to the heterogeneous Fischer-Tropsch process for the production of hydrocarbon fuels. C-C bond formation has been modeled by homogeneous systems, with [CO] fragments ( = 2-6) formed by two-electron reduction being commonly encountered. Here, we show that four- or six-electron reduction of CO can be accomplished by the use of anionic aluminum(I) ("aluminyl") compounds to give both topologically linear and branched C/C chains.

Structural Snapshots in Reversible Phosphinidene Transfer: Synthetic, Structural, and Reaction Chemistry of a Sn═P Double Bond.

The reaction of amido-substituted stannylenes with phospha-Wittig reagents (MePPR) results in release of hexamethyldisilazane and tethering of the resulting -CHPMePR fragment to the tin center to give P-donor stabilized stannylenes featuring four-membered ,,, heterocycles. Through systematic increases in steric loading, the structures of these systems in the solid state can be tuned, leading to successive P-P bond lengthening and Sn-P contraction and, in the most encumbered case, to complete P-to-Sn transfer of the phosphinidene fragment. The resulting stannaphosphene features a polar Sn═P double bond as determined by structural and computational studies.

Reaction of a bis(pentafulvene)titanium complex with an N-heterocyclic olefin: C-H-activation leads to resonance between a titanium vinyl and titanium alkylidene complex.

The N-heterocyclic olefin (NHO) ImMeCH (2) (ImMeCH = (MeCNMe)CCH) was employed for the synthesis of the titanium complex 3 derived from an NHO ligand precursor. By reacting 2 with the bis(π-η:σ-η-pentafulvene)titanium complex 1a, the terminal ylidic methylene unit of 2 is deprotonated by the quaternary exocyclic carbon atom of one pentafulvene ligand of 1a yielding the titanium complex 3 which bears an anionic NHO ligand (ImMeCH). 3 was characterized by NMR spectroscopy, single crystal X-ray diffraction and quantum chemical calculations.

Predicting the efficiency of oxygen-evolving electrolysis on the Moon and Mars.

Establishing a permanent human presence on the Moon or Mars requires a secure supply of oxygen for life support and refueling. The electrolysis of water has attracted significant attention in this regard as water-ice may exist on both the Moon and Mars. However, to date there has been no study examining how the lower gravitational fields on the Moon and Mars might affect gas-evolving electrolysis when compared to terrestrial conditions.

Generation of a π-Bonded Isomer of [P ] by Aluminyl Reduction of White Phosphorus and its Ammonolysis to PH.

By employing the highly reducing aluminyl complex [K{(NON)Al}] (NON=4,5-bis(2,6-diisopropylanilido)-2,7-di-tert-butyl-9,9-dimethylxanthene), we demonstrate the controlled formation of P and P complexes from white phosphorus, and chemically reversible inter-conversion between them. The tetra-anion features a unique planar π-bonded structure, with the incorporation of the K cations implicit in the use of the anionic nucleophile offering additional stabilization of the unsaturated isomer of the P fragment. This complex is extremely reactive, acting as a source of P : exposure to ammonia leads to the release of phosphine (PH ) under mild conditions (room temperature and pressure), which contrast with those necessitated for the direct combination of P and NH (>5 kbar and >250 °C).

Molecular Main Group Metal Hydrides.

This review serves to document advances in the synthesis, versatile bonding, and reactivity of molecular main group metal hydrides within Groups 1, 2, and 12-16. Particular attention will be given to the emerging use of said hydrides in the rapidly expanding field of Main Group element-mediated catalysis. While this review is comprehensive in nature, focus will be given to research appearing in the open literature since 2001.

Probing the Extremes of Covalency in M-Al bonds: Lithium and Zinc Aluminyl Compounds.

Synthetic routes to lithium, magnesium, and zinc aluminyl complexes are reported, allowing for the first structural characterization of an unsupported lithium-aluminium bond. Crystallographic and quantum-chemical studies are consistent with the presence of a highly polar Li-Al interaction, characterized by a low bond order and relatively little charge transfer from Al to Li. Comparison with magnesium and zinc aluminyl systems reveals changes to both the M-Al bond and the (NON)Al fragment (where NON=4,5-bis(2,6-diisopropylanilido)-2,7-di-tert-butyl-9,9-dimethylxanthene), consistent with a more covalent character, with the latter complex being shown to react with CO via a pathway that implies that the zinc centre acts as the nucleophilic partner.

A Stable Homoleptic Divinyl Tetrelene Series.

The synthesis of the new bulky vinyllithium reagent ( IPr=CH)Li, ( IPr=[(MeCNDipp) C]; Dipp=2,6-iPr C H ) is reported. This vinyllithium precursor was found to act as a general source of the anionic 2σ, 2π-electron donor ligand [ IPr=CH] . Furthermore, a high-yielding route to the degradation-resistant Si precursor IPr⋅SiBr is presented.

A vinyl silylsilylene and its activation of strong homo- and heteroatomic bonds.

The facile synthesis of a rare two-coordinate acyclic silylene (RSi:) that is stabilized using a bulky vinylic -heterocyclic olefin ligand and the strongly σ-donating hypersilyl group [Si(SiMe)] is reported. This vinyl-substituted silylene exhibits an excellent combination of prolonged thermal stability along with high reactivity towards small molecules. Despite being stable for months in solution, the reactivity of this new silylene is manifest in its ambient temperature activation of strong B-H, Si-Cl, C-O, P-P and C-H bonds.

Linking Low-Coordinate Ge(II) Centers via Bridging Anionic N-Heterocyclic Olefin Ligands.

We introduce a large-scale synthesis of a sterically encumbered N-heterocyclic olefin (NHO) and illustrate the ability of its deprotonated form to act as an anionic four-electron bridging ligand. The resulting multicenter donating ability has been used to link two low oxidation state Ge(II) centers in close proximity, leading to bridging Ge-Cl-Ge and Ge-H-Ge bonding environments supported by GeC heterocyclic manifolds. Reduction of a dimeric [RGeCl] species (R = anionic NHO, [(MeCNDipp)C═CH]; Dipp = 2,6-PrCH) did not give the expected acyclic RGeGeR analogue of an alkyne, but rather ligand migration/disproportionation transpired to yield the known diorganogermylene RGe and Ge metal.

Neutral, Cationic and Hydride-substituted Siloxygermylenes.

The introduction of the labile trimethylsiloxy group to Ge centers in the presence of an N-heterocyclic carbene donor is reported. The new complex IPr⋅GeCl(OSiMe ) (IPr=[(HCNDipp) C:]; Dipp=2,6-iPr C H ) was readily converted into the structurally unique Ge siloxy(hydrido)germylene IPr⋅GeH(OSiMe )⋅BH by treatment with lithium borohydride. Additionally, the reactive siloxygermylene cation [IPr⋅Ge(OSiMe )] was synthesized and clean oxidative addition of CH Cl was demonstrated.

Blood pressure variability predicts adverse events and cardiovascular outcomes in SPRINT.

SPRINT (Systolic Blood Pressure Intervention Trial) highlighted the benefits of intensive targeted antihypertensive therapy but resulted in higher rates of treatment-related adverse events. Blood pressure (BP) variability has emerged as a significant predictor of outcomes over and above levels of BP. Using the SPRINT data set, we aimed to determine the relationship of BP variability with cardiovascular outcomes and side effects of antihypertensive therapy.

An unusual flare of anti-synthetase syndrome during concurrent trastuzumab therapy given for recurrent breast cancer.

We present the case of a patient with relapsing anti-synthetase syndrome (ASS) that may have been triggered by monoclonal antibody trastuzumab therapy given for breast cancer. A 52-year-old female with a history of anti-Jo1-associated ASS went into remission with glucocorticoids and mycophenolate mofetil. Her past history included invasive ductal carcinoma of the right breast that was fully treated six years prior to the onset of ASS.

Approaching monocoordination at a silver(i) cation.

The recently reported bulky N-heterocyclic carbene ITr (ITr = [(HCNCPh)C:]) was found to stabilize low-coordinate Ag(i) environments. These electrophilic species were crystallographically identified as the weak solvates [(ITr)Ag(sol)] (sol = PhF, MesH or CHCl) and as a solvent-free dimer [(ITr)Ag]. The highly electrophilic nature of the [(ITr)Ag] cation was further demonstrated by the calculation of a very high methyl ion affinity (MIA) and the synthesis of [(ITr)Ag(PCO)] which features substantial side-on electron donation from a P-C π bond to Ag.

Pushing Chemical Boundaries with N-Heterocyclic Olefins (NHOs): From Catalysis to Main Group Element Chemistry.

N-Heterocyclic olefins (NHOs) have gone from the topic of a few scattered (but important) reports in the early 1990s to very recently being a ligand/reagent of choice in the far-reaching research fields of organocatalysis, olefin and heterocycle polymerization, and low oxidation state main group element chemistry. NHOs are formally derived by appending an alkylidene (CR) unit onto an N-heterocyclic carbene (NHC), and their pronounced ylidic character leads to high nucleophilicity and soft Lewis basic character at the ligating carbon atom. These olefinic donors can also be structurally derived from imidazole, triazole, and thiazole-based heterocyclic carbenes and, as a result, have highly tunable electronic and steric properties.

Accessing Low-Valent Inorganic Cations by Using an Extremely Bulky N-Heterocyclic Carbene.

The extremely bulky N-heterocyclic carbene (NHC), ITr (ITr=[(HCNCPh ) C:]) featuring sterically shielding umbrella-shaped trityl (CPh ) substituents was prepared. This NHC features the highest percent buried volume (%V ) to date, and was used to form a thermally stable quasi one-coordinate thallium(I) cation [ITr-Tl] . This Tl adduct and the corresponding lithium complex [ITr⋅Li(OEt )] are versatile "all-in-one" transmetalation/ligation reagents for preparing low-coordinate inorganic species inaccessible by pre-existing routes.