Practical Recommendations for the Diagnosis and Management of Lysosomal Acid Lipase Deficiency with a Focus on Wolman Disease.

Lysosomal acid lipase deficiency (LAL-D) is an ultra-rare lysosomal storage disease with two distinct phenotypes, an infantile-onset form (formerly Wolman disease) and a later-onset form (formerly cholesteryl ester storage disease). The objective of this narrative review is to examine the most important aspects of the diagnosis and treatment of LAL-D and to provide practical expert recommendations. The infantile-onset form occurs in the first weeks of life and is characterized by malnourishment and failure to thrive due to gastrointestinal impairment (vomiting, diarrhea, malabsorption), as well as systemic inflammation, hepatosplenomegaly, and adrenal calcifications.

Wine bioacidification: Fermenting Airén grape juices with Lachancea thermotolerans and Metschnikovia pulcherrima followed by sequential Saccharomyces cerevisiae inoculation.

Biological acidification and bioprotection are interesting tools to overcome some problems of climate change and fit some consumer requirements for fresher wine styles. Juices of Airén (Vitis vinifera L.) were fermented by Lachancea thermotolerans (Lt) and Metschnikowia pulcherrima (Mp) starters and compared with Saccharomyces cerevisiae (Sc).

Development of a model to study browning caused by tyrosinase in grape must.

Enzymatic browning caused by polyphenol oxidases, tyrosinase and laccase, continues to be one of the main problems in winemaking. Therefore, wineries are very interested in studying the mechanisms of browning and procedures for decreasing the use sulphur dioxide. This research proposes a model to study tyrosinase activity from grape must using different substrates: one monophenol (p-hydroxybenzoic acid), two diphenols (caftaric acid and (-)-epicatechin) and one triphenol (gallic acid).

Conventional management has a greater negative impact on L. rhizobia diversity and abundance than water scarcity.

Introduction: Drought is one of the biggest problems for crop production and also affects the survival and persistence of soil rhizobia, which limits the establishment of efficient symbiosis and endangers the productivity of legumes, the main source of plant protein worldwide.

Aim: Since the biodiversity can be altered by several factors including abiotic stresses or cultural practices, the objective of this research was to evaluate the effect of water availability, plant genotype and agricultural management on the presence, nodulation capacity and genotypic diversity of rhizobia.

Method: A field experiment was conducted with twelve common bean genotypes under irrigation and rain-fed conditions, both in conventional and organic management.