Adult-onset vanishing white matter disease due to a novel compound heterozygous EIF2B2 mutation: a case report and brief review.

Background And Objectives: Vanishing white matter disease (VWMD) is an autosomal recessive leukoencephalopathy caused by mutations in the EIF2B1-5 genes, typically rare in adulthood. We present a case of adult-onset VWMD with a novel EIF2B2 mutation.

Methods: We collected the patient's clinical data, cerebrospinal fluid (CSF) results, laboratory tests, imaging features, genetic analysis, and follow-up data over a 4-year period.

Modification of hordein by gallic acid in ethanol-free environments: Impact of covalent and non-covalent interactions on structure, physicochemical properties and self-assembly.

The objectives of this study were to modify hordein with gallic acid (GA) in alcohol-free media and to compare the impact of covalent and non-covalent binding on the properties of hordein. Covalent hordein-GA complexes (H-GA) and non-covalent hordein/GA complexes (H/GA) were distinguished by molecular weight, free sulfhydryl groups and free amino groups. Isothermal titration calorimetry (ITC) demonstrated that physical mixing induced non-covalent binding of GA to hordein via hydrogen bonding and hydrophobic interactions, with a lower binding efficiency than covalent ones.

Preparation of core-shell hordein/pectin nanoparticles as quercetin delivery matrices: Physicochemical properties and colon-specific release analyses.

Quercetin (Que) is a hydrophobic flavanol that has the potential to prevent colon diseases. This study aimed to design hordein/pectin nanoparticle as a colon-specific delivery system for quercetin. The encapsulation efficiency, physicochemical stability and release properties of the nanoparticles were estimated.

Different photoprotective strategies for white leaves between two co-occurring Actinidia species.

At the outer canopy, the white leaves of Actinidia kolomikta can turn pink but they stay white in A. polygama. We hypothesized that the different leaf colors in the two Actinidia species may represent different photoprotection strategies.

Two Nomograms to Select Hepatocellular Carcinoma Patients with Macroscopic Vascular Invasion for Hepatic Resection.

: Hepatocellular carcinoma (HCC) patients with macroscopic vascular invasion (MaVI) have limited lifespans. According to recent studies, surgical treatment may be the most promising option. However, the current staging system does not select patients who will benefit most from hepatic resection.

Dispersive liquid-liquid microextraction of silver nanoparticles in water using ionic liquid 1-octyl-3-methylimidazolium hexafluorophosphate.

Using the ionic liquid (IL) 1-octyl-3-methylimidazolium hexafluorophosphate as the extractant and methanol as the dispersion solvent, a dispersive liquid-liquid microextraction method was developed to extract silver nanoparticles (AgNPs) from environmental water samples. Parameters that influenced the extraction efficiency such as IL concentration, pH and extraction time were optimized. Under the optimized conditions, the highest extraction efficiency for AgNPs was above 90% with an enrichment factor of >90.