Earth-abundant MoS 2 is widely reported as a promising HER electrocatalyst in acidic solutions, but it exhibits extremely poor HER activities in alkaline media due to the slow water dissociation process. Here we present a combined theoretical and experimental approach to improve the sluggish HER kinetics of MoS 2 electrocatalysts through engineering the water dissociation sites by doping Ni atoms into MoS 2 nanosheets. The Ni sites thus introduced can effectively reduce the kinetic energy barrier of the initial water-dissociation step and facilitate the desorption of the À OH that are formed. As a result, the developed Ni-doped MoS 2 nanosheets (Ni-MoS 2 ) show an extremely low HER overpotential of B98 mV at 10 mA cm À2 in 1 M KOH aqueous solution, which is superior to those (4220 mV at 10 mA cm À2 ) of reported MoS 2 electrocatalysts.

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ObjectiveTo determine whether the GGC repeats in the NOTCH2NLC gene contribute to amyotrophic lateral sclerosis (ALS).MethodsIn this study, 545 ALS patients and 1,305 healthy controls from mainland China were recruited. Several pathogenic mutations in known ALS-causative genes (including C9ORF72 and ATXN2) and polynucleotide repeat expansions in NOP56 and AR genes were excluded. Repeat-primed PCR (RP-PCR) and GC-rich PCR were performed to determine the GGC repeat size in NOTCH2NLC. Systematic and targeted clinical evaluations and investigations, including skin biopsy and dynamic electrophysiologic studies, were conducted in the genetically affected patients.ResultsGGC repeat expansion was observed in 4 patients (numbers of repeats: 44, 54, 96, and 143), accounting for approximately 0.73% (4/545) of all ALS patients. A comparison with 1,305 healthy controls revealed that GGC repeat expansion in NOTCH2NLC was associated with ALS (Fisher's exact test, 4/545 vs 0/1,305, p = 0.007). Compared to patients with the neuronal intranuclear inclusion disease (NIID) muscle-weakness-dominant subtype, patients with ALS phenotype carrying the abnormal repeat expansion tended to have a severe phenotype and rapid deterioration.ConclusionOur results suggest that ALS is a specific phenotype of NIID or that GGC expansion in NOTCH2NLC is a factor that modifies ALS. These findings may help clarify the pathogenic mechanism of ALS and may expand the known clinical spectrum of NIID.