Alkali-assisted extraction, characterization and encapsulation functionality of enzymatic hydrolysis-resistant prolamin from distilled spirit spent grain.

Curcumin is a natural lipophilic polyphenol that exhibits significant various biological properties such as antioxidant and anti-inflammatory properties following oral administration. However, its uses have shown limitations concerning aqueous solubility, bioavailability and biodegradability that could be improved by prolamin-based nanoparticle. In this study, curcumin was encapsulated into prolamin from sorghum (SOP) and wheat (WHP) and distilled spirit spent grain (DSSGP), which was obtained after microbial proteolysis of the former two cereal grains. All the three prolamins showed clear variation of protein profiles and microstructure as confirmed by electrophoresis analysis, disulfide bond determination and Fourier-transform infrared spectroscopy (FTIR). For curcumin-loaded nanospheres (NPs) fabrication, three prolamin-based NPs shared features of spherical shape, uniform particle size, and smooth surface. The average size ranged from 122 to 193 nm depending on the prolamin variety and curcumin loading. In the experiments in vitro, curcumin showed significantly improved UV/thermal stability. Furthermore, DSSGP was more resistant to enzymatic digestion in vitro, hence achieving the controlled release of curcumin in gastrointestinal tract. Collectively, the results indicated the improved bioavailability and biodegradability of curcumin encapsulated by DSSGP, which would be an innovative potential encapsulant for effective protection and targeted delivery of hydrophobic compounds.

Lentinula edodes Sing Polysaccharide: Extraction, Characterization, Bioactivities, and Emulsifying Applications.

In the present work, the optimization of extraction, emulsifying properties, and biological activities of polysaccharides from Lentinula edodes Sing (LES) were studied. The results showed LES polysaccharides extracted by hot water or ultrasonication are a group of ß-glucan. Among all the samples, the one extracted by hot water showed the best emulsifying capacity. In addition, the results demonstrated that LES polysaccharide had strong scavenging activities in vitro on DPPH and ABTS radicals, which reached the highest level for the one extracted by 90 min ultrasonication (p < 0.05). Overall, Lentinula edodes Sing polysaccharides (LESPs) may have potential applications as emulsifying agents in food industries.

Effect of weekend versus weekday admission on the mortality of acute ischemic stroke patients in China: an analysis of data from the Chinese acute ischemic stroke treatment outcome registry.

Background: Due to disparities in medical resources in rural and urban areas as well as in different geographic regions in China, the effect of weekend versus weekday admission on the outcomes of acute ischemic stroke (AIS) patients is unknown. Our aim was to investigate whether the outcomes of AIS patients differ according to the day of admission in China. Methods: The data were extracted from the Chinese Acute Ischemic Stroke Treatment Outcome Registry (CASTOR), a multicenter prospective study database of patients diagnosed with AIS. The chi-square test (χ2) and logistic regression were used to assess mortality for weekday and weekend admissions among AIS patients stratified by rural or urban status and geographic region (including the eastern, northeastern, central, and western regions). Results: In total, 9,256 patients were included in this study. Of these patients, 57.2% were classified as urban, and 42.8% were classified as rural. A total of 6,760 (73%) patients were admitted on weekdays, and 2,496 (27%) were admitted on weekends. There was no significant difference in the mortality rate among patients admitted on weekends compared with those admitted on weekdays in urban (7.5% versus 7.4%) or rural areas (8.8% versus 8.1%; p > 0.05). The mortality rate was the highest among patients admitted on weekends and weekdays (11.6% versus 10.3%) in the northeastern area, without statistical significance before and after adjusting for the patients' background characteristics (p > 0.05). In addition, regression analysis revealed that the mortality of patients admitted on weekdays was more likely to be influenced by regional subgroup, hospital level and intravenous thrombolysis than that of patients admitted on weekends. Conclusion: The weekend effect was not observed in the mortality of patients with AIS regardless of rural-urban status or geographic region in China.

Evaluation of the median nerve by shear wave elastography in patients with Charcot-Marie-Tooth disease type 1A.

AIMS: Charcot-Marie-Tooth disease type 1A (CMT1A) is characterized by enlargement and stiffness of peripheral nerves due to edema with large numbers of "onion bulbs" in the endoneurium. Ultrasound elastography seems to be an ideal method to detect this condition. The aim of this study was to analyze the shear wave elastography (SWE) features of peripheral nerves in patients with CMT1A. MATERIAL AND METHODS: We included 24 CMT1A patients with a mean age of 28 years, along with 24 age- and gender-matched controls. All patients presented with mutations of the PMP22 gene and showed length-dependent polyneuropathy. The motor nerve conduction velocity (MNCV) of the median nerve ranged from 5.2 to 37.4 m/s. SWE and cross-sectional area (CSA) were used to evaluate the bilateral median nerves at predefined sites in both patients and con-trols. RESULTS: The average elastography value (EV) of the median nerve was 73.5±11.7 kPa in patients with CMT1A and 37.5±6.1 kPa in control subjects. The difference between the two groups was statistically significant (P<0.05). In CMT1A pa-tients, the average EV at the proximal and distal parts of the median nerve were 81.4±9.4 kPa and 65.2±8.1 kPa, respectively. The average CSAs at the proximal and distal parts of the median nerve were 0.29±0.06 cm2 and 0.20±0.05 cm2, respectively. The EV on SWE was positively correlated with CSA (p< 0.01) and negatively correlated with MNCV in the median nerve (p< 0.01). CONCLUSIONS: Peripheral nerve stiffness dramatically increases in CMT1A and is correlated with the severity of nerve involvement.

Pathogenic PSAT1 Variants and Autosomal Recessive Axonal Charcot-Marie-Tooth Disease With Ichthyosis.

BACKGROUND: Biallelic pathogenic phosphoserine aminotransferase 1 (PSAT1) variants generally cause a severe phenotype predominantly involving the central nervous system. Here, for the first time, we report two patients harboring pathogenic PSAT1 variants only manifested as polyneuropathy and ichthyosis. METHODS: Two patients from unrelated families presenting with polyneuropathy and ichthyosis were enrolled. Whole exome sequencing was performed to identify possible disease-causing variants. Their clinical, electrophysiological, imaging, biochemical, and pathologic changes were in detail assessed and investigated. RESULTS: Homozygous variant c.43G>C and compound heterozygous variants c.112A>C and c.43G>C in PSAT1 were identified in patients 1 and 2, respectively. Nerve conduction studies revealed preserved or mild slowing motor nerve conduction velocities of the median nerves in the two patients, whereas the compound motor action potential in patient 1 was severely decreased. Brain magnetic resonance imaging of the two patients found no abnormalities. Median nerve enlargement was observed on ultrasound in patient 1. Both patients had normal level of serine and glycine in plasma and cerebrospinal fluid. Sural nerve biopsy found severe loss of myelinated fibers. Electron microscopy revealed neurofilament accumulation and mitochondrial aggregation in axons. Both variants in PSAT1 were classified as likely pathogenic or pathogenic variants according to the standard guidelines. CONCLUSIONS: Our study confirms that pathogenic PSAT1 variants can cause a mild phenotype, predominantly as autosomal recessive axonal Charcot-Marie-Tooth disease.

Clinicopathological features in two families with MARS-related Charcot-Marie-Tooth disease.

Mutations in MARS gene cause dominant Charcot-Marie-Tooth disease (CMT) 2U. The aim of this study is to investigate phenotypic heterogeneities and peripheral neuropathology of MARS-related CMT patients. We identified a heterozygous p. R199Q mutation and an already reported heterozygous p. P800T mutation of MARS gene in two unrelated families using targeted next-generation sequencing. The first pedigree comprised three patients over three generations and the second pedigree comprised two patients over two generations. In addition of an asymptomatic carrier in the second pedigree, all patients presented with childhood-onset length dependent sensorimotor neuropathy with pes cavus. Nerve conduction studies revealed slowing of motor nerve conduction velocities (MNCV) of the median nerve indicating intermediate neuropathy in the patient with the p. R199Q mutation, and normal MNCV with reduced compound muscle action potential indicating axonal neuropathy in the patient with the p. P800T mutation. Magnetic resonance imaging detected a pattern of nerve changes similar to those in demyelinating polyneuropathies in intermediate type (p. R199Q mutation) patients compared with normal in the axonal type (p. P800T mutation) patients. Additionally, sural nerve biopsy revealed loss of myelinated axons with onion bulb formation in both mutations. By electron microscopy, a marked decrease of myelinated and unmyelinated fiber, neurofilaments aggregate with degenerating mitochondria and microtubule loss in axons were frequently found. Denervated Schwann cell complexes and few collagen pockets indicated involvement of unmyelinated Schwann cells. Therefore, the investigated MARS mutations cause not only the known axonal type but also intermediate type neuropathy with involvement of both axons and Schwann cells. Those findings are useful for the differential diagnosis of CMT patients with unknown MARS variants.

Reduced perfusion in normal-appearing white matter in mild to moderate hypertension as revealed by 3D pseudocontinuous arterial spin labeling.

PURPOSE: To investigate the hemodynamic changes of normal-appearing white matter (NAWM) in hypertension using the 3D pseudocontinuous arterial spin labeling (pCASL) technique. MATERIALS AND METHODS: Seventy-three subjects, including a patient group (n = 41; 30 males; age = 47.7 ± 8.3 years; test-time blood pressure [BP] = 155 ± 23/98 ± 11 mmHg) and an age-matched control group (n = 32; 14 males; age = 46 ± 8.3 years; test-time BP = 117 ± 8/76 ± 10 mmHg), were recruited and scanned on a 3.0T magnetic resonance imaging (MRI) system using routine MRI sequences and 3D pCASL sequence. The routine MRI sequences were used to further define the NAWM. The cerebral blood flow (CBF) values in various regions of interest (ROIs) were extracted. One-way analysis of variance (ANOVA) and unpaired t-test were performed to evaluate the significance of the intergroup difference in CBF modifications. RESULTS: Compared to healthy volunteers, CBF values in global gray matter (GM) and various NAWM regions were found to be lower (P < 0.05) in hypertensive patients, except for genu of corpus callosum (CC), cingulate gyrus, amygdala, pallidum, putamen, and thalamus (P > 0.05). Furthermore, compared to the control group, mild hypertension showed significantly reduced CBF in various ROIs (P < 0.05), but no intergroup differences in GM, R anterior horn of periventricular WM, and genu of CC (P > 0.05), while moderate hypertension showed reduced CBF in all ROIs (P < 0.05). However, it was observed that, between mild and moderate hypertensive patients, there were no statistically significant difference in CBF values except for genu of CC (P < 0.05). CONCLUSION: 3D pCASL has the ability to detect subtle hemodynamic abnormalities in NAWM regions at relatively early stages of hypertension. The observed decreases in CBF in these regions may suggest an increased risk of cerebral small vessel diseases.

Reliability of three-dimensional pseudo-continuous arterial spin labeling MR imaging for measuring visual cortex perfusion on two 3T scanners.

Cerebral blood flow (CBF) in the human primary visual cortex is correlated with the loss of visual function in neuro-ophthalmological diseases. Advanced three-dimensional pseudo-continuous arterial spin labeling (3D pCASL), as a non-invasive method to access the CBF, can be a novel measurement to detect the visual cortex. The objective of the study was to assess the intra- and inter-scanner reliability of 3D pCASL of the visual cortex in healthy adults and suggest the selection of different post-labeling delay times (PLDs). For this reason, 3D pCASL was conducted in two 3.0T MR three times with twelve healthy volunteers at an interval of 10-15 days. The 1st and 3rd tests were performed on scanner-1, and the 2nd test was performed on scanner-2. The value of the CBF was abstracted from the visual cortex with two PLDs. The intra- and inter-scanner reliability and reproducibility were evaluated with the intraclass correlation coefficient (ICC) and Bland-Altman plots. By estimating the mean value of the CBF in the visual cortex, the intra-scanner results demonstrated the higher reliability (ICC for PLD = 1.5 second presented at 0.743 compared with 0.829 for PLD = 2.5 seconds), and the Bland-Altman plots showed the reproducibility at a longer PLD. We conclude that the calibrated 3D pCASL approach provides a highly reproducible measurement of the CBF of the visual cortex that can serve as a useful quantitative probe for research conducted at multiple centers and for the long-term observation of the clinical effects of neuro-opthalmological diseases.