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Lysosomal acid lipase (LAL) is a serine hydrolase that hydrolyzes cholesteryl ester (CE) and TGs delivered to the lysosomes into free cholesterol and fatty acids. LAL deficiency due to mutations in the LAL gene (LIPA) results in accumulation of TGs and cholesterol esters in various tissues of the body leading to pathological conditions such as Wolman's disease and CE storage disease (CESD). Here, we present the first crystal structure of recombinant human LAL (HLAL) to 2.6 Å resolution in its closed form. The crystal structure was enabled by mutating three of the six potential glycosylation sites. The overall structure of HLAL closely resembles that of the evolutionarily related human gastric lipase (HGL). It consists of a core domain belonging to the classical α/β hydrolase-fold family with a classical catalytic triad (Ser-153, His-353, Asp-324), an oxyanion hole, and a “cap” domain, which regulates substrate entry to the catalytic site. Most significant structural differences between HLAL and HGL exist at the lid region. Deletion of the short helix, 238NLCFLLC244, at the lid region implied a possible role in regulating the highly hydrophobic substrate binding site from self-oligomerization during interfacial activation. We also performed molecular dynamic simulations of dog gastric lipase (lid-open form) and HLAL to gain insights and speculated a possible role of the human mutant, H274Y, leading to CESD.

**Background:** Chorea is recognized as a prototypic motor feature of Huntington’s disease (HD), but its effect on health-related quality of life (HRQoL) has not been fully explored. This study describes the impact of chorea on HRQoL in patients with HD. **Objective:** To determine the impact of HD-related chorea on employment, self-care activities, activities of daily living, and health-care resource utilization (HCRU). **Methods:** Data were drawn from the Adelphi HD Disease Specific Programme, a real-world point-in-time survey of 144 neurologists and 427 patients in the United States between July and October 2017. HD patients with and without chorea were identified and examined for differences in employment status, reasons for employment changes, self-care activities, and modifications to cope with involuntary movements. Bivariate tests and inverse probability weighted regression adjustment methods were used to determine differences in outcomes between patients with and without chorea. **Results:** HD patients with (n=287) and without (n=140) chorea were identified. Patients with chorea were less likely to be employed full-time (16.7% vs 25.7%; _P_

Hierarchical Ni(OH)2/Co(OH)2 nanocomposite were produced by the use of hydrothermal technique for sustainable alternative energy production and employed as competent electrocatalyst for oxygen evolution reaction (OER). Herein, prepared electrocatalyst achieved 3D urchin sphere like morphology at the scale range of 1 μm and 200 nm. Electrochemical studies of Ni(OH)2/Co(OH)2 nanocomposite was analyzed by 3 electrode using aqueous alkaline solution of 1 M KOH. Prepared Ni(OH)2/Co(OH)2 nanocomposite electrode attained 264.5 cm2 electrochemical surface area obtained from high double layer capacitance (Cdl) value of 10.58 mF/cm2 and it required very low 360 mV at 50 mA/cm2 current density with 240 mV/dec Tafel value which is distinctly favorable for the effective electrocatalytic activity. The low solution resistance (Rs) value of 0.54 Ω showed the excellent conductivity and charge transfer competence. The prepared product displays good stability and the consistency up to the long duration of 16 h at 10 mA/cm2. Furthermore, morphological stability of prepared nanocomposite electrode was also confirmed from the post SEM analysis which concluded the significant impact on structural transformation of nanocomposite after the electrochemical analysis. Present work revealed better perceptive of benefits and defects of alternative low cost Ni(OH)2/Co(OH)2 nanocomposite electrocatalyst for water oxidation process. Hence, synthesized Ni(OH)2/Co(OH)2 explored good electrocatalyst for OER and open new way to design alternative low cost electrocatalytic materials for sustainable energy generation. Highlights: Ni(OH)2/Co(OH)2 electrode attained 264.5 cm2 electrochemical surface area. Obtained high double layer capacitance (Cdl) value of 10.58 mF/cm2. Achieved very low 360 mV at 50 mA/cm2 current density with 240 mV/dec Tafel value. Low 0.54 Ω solution resistance (Rs) showed excellent conductivity. Displayed good stability and consistency up to long duration of 16 h at 10 mA/cm2. [ABSTRACT FROM AUTHOR]