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Δευτέρα 18 Φεβρουαρίου 2019

Depression: An Overlooked Villain in Autoimmune Hepatitis?

Abstract

We read with great interest the recent article by Wong et al. on the impact of autoimmune hepatitis (AIH) on health‐related quality of life (HRQoL).(1) So far, this issue has been vastly neglected in patients with AIH. Our particular attention has been drawn to data on mood disturbance, particularly depression. This problem is of great importance in patients with AIH, as depression is one of the key factors responsible for nonadherence to therapy, which increases the risk of relapse and disease progression. A considerable proportion of AIH patients are young adults, often on prolonged steroids that are shown to significantly affect mood.

This article is protected by copyright. All rights reserved.



https://aasldpubs.onlinelibrary.wiley.com/doi/abs/10.1002/hep.30568?af=R

Repair or Prevent – What is the real impact of normothermic machine perfusion in liver transplantation?

Abstract

We read with great interest the paper by Jassem et al(1), which provides detailed gene expression profiles of human livers during normothermic machine perfusion (NMP), compared to conventional cold storage. Authors should be congratulated to this extensive analysis, which shows for the first time transcriptional changes during any form of reperfusion under normothermic conditions(1). However, we would like to add a few critical comments.

This article is protected by copyright. All rights reserved.



https://aasldpubs.onlinelibrary.wiley.com/doi/abs/10.1002/hep.30567?af=R

Production and characterization of a new glycolipid, mannosylerythritol lipid, from waste cooking oil biotransformation by Pseudozyma aphidis ZJUDM34

Food Science & Nutrition Production and characterization of a new glycolipid, mannosylerythritol lipid, from waste cooking oil biotransformation by Pseudozyma aphidis ZJUDM34

The production of MELs using waste cooking oil instead of soybean oil was successful, and the major characteristics and compositions of MELs were also investigated. The use of inexpensive fermentation substrates associated with straightforward downstream processing is expected to have a great impact on the economy of MEL production.


Abstract

Mannosylerythritol lipids (MELs) are glycolipids possessing unique biosurfactant properties. However, the prices of substrates currently used for MEL formation caused its unsustainable commercial development. Waste cooking oil poses significant ecological and economical problems. Thus, the production of MELs from used waste cooking oil using the biotransformation route is one of the better alternatives to utilize it efficiently and economically. This work aims at the production of MELs using waste cooking oil instead of soybean oil and evaluating the major characteristics and compositions of MELs. The titers reached 61.50 g/L by the optimization of culture medium, higher than the counterpart (10.25 ± 0.32 g/L) of the nonoptimized medium. MELs exhibited good surface activity and better performance in contrast to MELs grown on soybean oil. The water phase behavior of MEL‐A was also evaluated. The process showed higher productivity of MELs with better surface activity and application stability than the conventional process using soybean oil. The findings of this study imply that the use of inexpensive fermentation substrates associated with straightforward downstream processing is expected to have a great impact on the economy of MEL production.



https://onlinelibrary.wiley.com/doi/abs/10.1002/fsn3.880?af=R

Royal jelly causes hypotension and vasodilation induced by increasing nitric oxide production

Food Science & Nutrition Royal jelly causes hypotension and vasodilation induced by increasing nitric oxide production

RJ has antihypertensive effects and is associated with increased NO production. In addition, RJ contains muscarinic receptor agonists and induces vasorelaxation through NO/cGMP pathway and calcium channels.


Abstract

Among royal jelly's (RJ) various biological activities, its possible antihypertension and vasorelaxation effects deserve particular attention, but the underlying mechanisms of action remain unclear. Therefore, this study used the spontaneously hypertensive rats (SHR) hypertension model and the isolated rabbit thoracic aorta rings model to explore the mechanisms underlying the hypotension and vasorelaxation effects of RJ. Rats were divided into the following groups (n = 6): WKY‐control group, SHR‐control group, and SHR‐RJ group. SHR‐RJ group was received 1 g/kg of RJ via oral administration daily for 4 weeks. Systolic blood pressure (SBP), diastolic blood pressure (DBP), heart rate (HR), and nitric oxide (NO) level were detected. In addition, the mechanism of vasodilation of RJ was investigated using an isolated rabbit aortic ring technique. RJ significantly reduced SBP and DBP as well as increased NO levels of SHR in vivo. RJ caused vasorelaxation of the isolated aorta rings, and this effect was inhibited by atropine (M3 receptor blocker), L‐NAME (nitric oxide synthase inhibitor), methylene blue (guanylate cyclase inhibitor), and indomethacin (cyclooxygenase inhibitor). Moreover, RJ could markedly suppress the NE‐induced intracellular Ca2+ releases and high K+‐induced extracellular Ca2+ influx in denuded aortic rings. In addition, RJ can also increase cGMP levels and the production of NO in isolated aortic rings. The present study showed that RJ has antihypertensive effects and was associated with increased NO production. In addition, RJ contains muscarinic receptor agonist, possibly an acetylcholine‐like substance, and induces vasodilation through NO/cGMP pathway and calcium channels.



https://onlinelibrary.wiley.com/doi/abs/10.1002/fsn3.970?af=R

Alteration of the lipid of red carp (Cyprinus carpio) during frozen storage

Food Science & Nutrition Alteration of the lipid of red carp (Cyprinus carpio) during frozen storage

Lipid oxidation of red carp fish during frozen storage was assessed by FTIR method. Druring storage, peroxide acid increased, while unsaturated fatty acids and triglycerides contents decreased. these decreased the nutritional value of red carp.

Fish analysis


Abstract

The aim of this study was to determine the oxidative stability of oil extracted from red carp fish frozen up to 9 months at −18°C. To assess oil stability of red carp fish, the analytical indexes and Fourier transform infrared (FTIR) spectroscopy were used. These methodologies used provided similar conclusions. Before frozen storage, the composition of fatty acids showed that red carp oil is a good source of polyunsaturated fatty acids (PUFAs) such as linoleic acid (C18:2ω‐6: 5.29% of total fatty acid), linolenic acid (C18:3ω3: 3.53% of total fatty acid), arachidonic acid (C20:4ω6: 3.68% of total fatty acid), eicosapentaenoic acid (C20:5ω‐3, EPA: 4,06% of total fatty acid), and docosahexaenoic acid (C22:6ω‐3: 3.02% of total fatty acid). During frozen storage, the free fatty acid and peroxide value increased, respectively, from 1.35% to 8.06% in oleic acid and 3.77 to 18.62 meq O2/kg in lipid, while the ratio of PUFA/SFA and polyene index decreased, respectively, from 0.58 to 0.25 and 0.30 to 0.09. The triglycerides also decreased with frozen duration. Therefore, for good fish quality, red carp fish must be stored for <3 months at −18°C.



https://onlinelibrary.wiley.com/doi/abs/10.1002/fsn3.971?af=R

Homozygous variant in OTX2 and possible genetic modifiers identified in a patient with combined pituitary hormone deficiency, ocular involvement, myopathy, ataxia, and mitochondrial impairment

Here we report on a singleton patient affected by a complicated congenital syndrome characterized by growth delay, retinal dystrophy, sensorineural deafness, myopathy, ataxia, combined pituitary hormone deficiency, associated with mitochondrial impairment. Targeted clinical exome sequencing led to the identification of a homozygous missense variant in OTX2. Since only dominant mutations within OTX2 have been associated with cases of syndromic microphthalmia, retinal dystrophy with or without pituitary dysfunctions, this represents the first report of an OTX2 recessive mutation. Part of the phenotype, including ataxia, myopathy and multiple mitochondrial respiratory chain defects, seemed not related to OTX2. Further analysis of next generation sequencing (NGS) data revealed additional candidate variants: a homozygous variant in LETM1, and heterozygous rare variants in AFG3L2 and POLG. All three genes encode mitochondrial proteins and the last two are known to be associated with ataxia, a neurological sign present also in the father of the proband. With our study, we aim to encourage the integration of NGS data with a detailed analysis of clinical description and family history in order to unravel composite genotypes sometimes associated with complicated phenotypes.



https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.61092?af=R

Expanding the genetic and clinical spectrum of the NONO‐associated X‐linked intellectual disability syndrome

The NONO gene encodes a nuclear protein involved in RNA metabolism. Hemizygous loss‐of‐function NONO variants have been associated with syndromic intellectual disability and with left ventricular noncompaction (LVNC). A two‐year‐old boy presented to the University of Utah's Penelope Undiagnosed Disease Program with developmental delay, nonfamilial features, relative macrocephaly, and dilated cardiomyopathy with LVNC and Ebstein anomaly. Brain MRI showed a thick corpus callosum, mild Chiari I malformation, and a flattened pituitary. Exome sequencing identified a novel intronic deletion (c.154+5_154+6delGT) in the NONO gene. Splicing studies demonstrated intron 4 read‐through and the use of an alternative donor causing the frameshift p.Asn52Serfs*6. Family segregation analysis showed that the variant occurred de novo in the boy's unaffected mother. MRI and endocrine findings suggest that hypopituitarism may contribute to growth failure, abnormal thyroid hormone levels, cryptorchidism, or delayed puberty in patients with NONO‐associated disease. Also, including this case LVNC has been observed in five out of eight patients, and this report also confirms an association between loss of NONO and Ebstein anomaly. In some cases, unrelated individuals share the same pathogenic NONO variants but do not all have clinically significant LVNC, suggesting that additional modifiers may contribute to cardiac phenotypes.



https://onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.61091?af=R