Aim: It was aimed to identify markers that would indicate which cases presenting with rhabdomyolysis are more likely to be associated with inherited metabolic diseases. Methods: We analyzed 327 children who applied to our Hospital Pediatric Nutrition and Metabolic Diseases Clinic with rhabdomyolysis. The diagnosis of rhabdomyolysis was made by measuring the serum creatinine kinase level in cases presenting with muscle pain, weakness and dark urine. Results: Metabolic disease was detected in 29 (16/13, M/F) patients from 26 different families. 298 patients (165/133, M/F) had normal metabolic work-up. We detected glutaric aciduria type 2 in 13 patients (44,6%), glycogen storage disease type 5 in three patients (10,3%), MCAD deficiency in three patients(10,3%), mitochondrial disease in three patients (10,3%), glycogen storage disease type 9 in one patient (3,5%), VLCAD deficiency in one patient (3,5%), LCHAD deficiency in one patient (3,5%), CPT2 deficiency in one patient(3,5%), Tango2 deficiency in one patient (3,5%), lipin-1 deficiency in one patient (3,5%) and primary carnitine deficiency in one patient (3,5%). Conclusion: In our study, consanguineous marriage, developmental delay, and intellectual disability were found more frequently in patients with metabolic disease. In addition, CK levels above 2610 U/L was found to be significantly correlated with metabolic disease.

Postharvest disease caused by fungi is a major issue that leads to quality decline and economic losses in mango fruit during storage and distribution. TGA transcription factors and pathogenesis-related proteins (PR) play crucial roles in modulating plant tolerance to pathogens. However, the roles of TGAs in the disease resistance of mango fruit remain unclear. Here, we investigated the impact of applying the antagonist Bacillus siamensis (N-1) on disease occurrence and the expression levels of MiTGA1 and MiPR1 genes in “Tainong No. 1” mango. We also explored the molecular mechanism of MiTGA1 interaction with MiPR1 gene. Results demonstrated that N-1 treatment significantly increased the level of hydrogen peroxide (H2O2) and effectively suppressed disease expansion during mango storage at 25 °C. Analyses of transcriptome data and qRT-PCR revealed the obvious up-regulation of MiTGA1 and MiPR1 genes in response to the N-1 treatment. Further subcellular localization identified the MiTGA1 protein as being located in the nucleus. Yeast one-hybrid (Y1H) and dual-luciferase reporter (DLR) assays confirmed that MiTGA1 could bind to the MiPR1 promoter and activate its transcription. Furthermore, transient over-expression of MiTGA1 in mango was found to enhance the accumulation of disease-resistant substances such as H2O2 by modulating MiPR1 expression, thereby bolstering the disease resistance of mango fruit. Our study suggests that MiTGA1 is a promising target gene and its interaction with MiPR1 may contribute to disease resistance induction and decay mitigation in postharvest mango fruit.

The herbaceous peony (<i>Paeonia lactiflora</i> Pall.) flower is one of the most important ornamental plants in current international flower markets and is widely used for festive occasions because of its bright colors, large flowers, pleasant scent, and plump flower shape. However, the cut peony flowers have a relatively short postharvest life in vases compared to other flower species. The short vase life and susceptibility to gray mold disease caused by <i>Botrytis cinerea</i> significantly influence the commercial value of cut peonies. Here, we reviewed the main factors for postharvest quality reduction in cut peony flowers and the various postharvest treatments aiming for vase life extension and enhancing gray mold disease resistance in cut peonies. Chemical treatments, including ethylene inhibitors, antimicrobial agents, and other preservatives, have been assessed for their effectiveness in improving vase life. Moreover, physical treatments, such as temperature management, controlled atmosphere storage, and dry storage methods, were also discussed for their effectiveness in delaying flower senescence and reducing gray mold disease infection. The review also highlighted the importance of cultivar-specific responses to gray mold disease, ethylene, and water stress, which is important for the development of new effective and specific postharvest practices to improve the vase life of cut peonies.

Abstract Zinc metabolism at the cellular level is critical for many biological processes in the body. A key observation is the disruption of cellular homeostasis, often coinciding with disease progression. As an essential factor in maintaining cellular equilibrium, cellular zinc has been increasingly spotlighted in the context of disease development. Extensive research suggests zinc’s involvement in promoting malignancy and invasion in cancer cells, despite its low tissue concentration. This has led to a growing body of literature investigating zinc’s cellular metabolism, particularly the functions of zinc transporters and storage mechanisms during cancer progression. Zinc transportation is under the control of two major transporter families: SLC30 (ZnT) for the excretion of zinc and SLC39 (ZIP) for the zinc intake. Additionally, the storage of this essential element is predominantly mediated by metallothioneins (MTs). This review consolidates knowledge on the critical functions of cellular zinc signaling and underscores potential molecular pathways linking zinc metabolism to disease progression, with a special focus on cancer. We also compile a summary of clinical trials involving zinc ions. Given the main localization of zinc transporters at the cell membrane, the potential for targeted therapies, including small molecules and monoclonal antibodies, offers promising avenues for future exploration.

Wheat is one of the most significant crops commercially because of its growing use. There is a real concern about the safety of grain that has been stored for a long time on a worldwide scale in order to get and retain a supply of high-quality grains. There are numerous methods to store wheat, however, traditional techniques are still used in Algeria. During storage, wheat grains are infested with pests and diverse microbial populations that can cause grain loses and disease. Preharvest, harvest, transportation, storage, and processing are all potential sources of microbial contamination in wheat. Fungi are the most contaminating flora, presenting mycotoxin excretion risk. Whereas lactic acid bacteria represent the fermentation flora. Contrary to what some research has found regarding the decrease in the nutritional value of wheat during storage, the storage in underground pits can enhance the product’s nutritional quality. Durum wheat fermentation is primarily a crucial technique that, when done on an industrial scale, might produce new products with improved technological and nutritional qualities. This present review highlights the storage methods available worldwide, different losses of wheat during storage, the effects of the storage conditions and natural fermentation on the quality of the stored wheat.

Patulin is a secondary metabolite primarily synthesized by the fungus Penicillium expansum, which is responsible for blue mold disease on apples. The latter are highly susceptible to fungal infection in the postharvest stages. Apples destined to produce compotes are processed throughout the year, which implies that long periods of storage are required under controlled atmospheres. P. expansum is capable of infecting apples throughout the whole process, and patulin can be detected in the end-product. In the present study, 455 apples (organically and conventionally grown), destined to produce compotes, of the variety “Golden Delicious” were sampled at multiple postharvest steps. The apple samples were analyzed for their patulin content and P. expansum was quantified using real-time PCR. The patulin results showed no significant differences between the two cultivation techniques; however, two critical control points were identified: the long-term storage and the deck storage of apples at ambient temperature before transport. Additionally, alterations in the epiphytic microbiota of both fungi and bacteria throughout various steps were investigated through the application of a metabarcoding approach. The alpha and beta diversity analysis highlighted the effect of long-term storage, causing an increase in the bacterial and fungal diversity on apples, and showed significant differences in the microbial communities during the different postharvest steps. The different network analyses demonstrated intra-species relationships. Multiple pairs of fungal and bacterial competitive relationships were observed. Positive interactions were also observed between P. expansum and multiple fungal and bacterial species. These network analyses provide a basis for further fungal and bacterial interaction analyses for fruit disease biocontrol.

GM1 gangliosidosis (GM1) is a rare autosomal recessive neurogenerative lysosomal storage disease characterized by deficiency of beta-galactosidase (β-gal) and intralysosomal accumulation of GM1 ganglioside and other glycoconjugates. Resources for GM1 disease modelling are limited, and access to relevant cell lines from human patients is not possible. Generation of iPSC lines from GM1 patient-derived dermal fibroblasts allows for disease modelling and therapeutic testing in 2D and 3D cell culture models relevant to CNS disorders, including various neuronal subtypes and cerebral organoids. The iPSC line described here will be critical to therapeutic development and set the foundation for translational gene therapy work.

Anthracnose disease (Colletotrichum spp.) in chili can lead to low fruit quantity and quality from planting to the postharvest stage. The environmentally friendly management using chitosan (CS) is expected to suppress anthracnose disease and increase the shelf-life of chili. This study aimed to determine the effect of the chitosan-tripolyphosphate (CS-TPP) to suppress anthracnose on chili during postharvest storage through in-vitro and in-vivo assays. In the in-vitro assay, CS-TPP solution with a ratio of [5:2] and [3:1] was applied into a warm Potato Dextrose Agar (PDA) medium. It was grown by Colletotrichum pathogen, while on in vivo assay, CS-TPP was applied to chili fruit before being inoculated by the pathogen. A control treatment was prepared without CS-TPP application. Results revealed that the CS-TPP ratios affected the growth of Colletotrichum spp. at the in-vitro assay. The CS-TPP [5:2] ratio was more effective than CS-TPP [3:1] in reducing the growth of Colletotrichum spp. with the fungal inhibition of 62,65% and 55,56%, respectively, compared to the control treatment. Moreover, it also showed anthracnose disease suppression on chili fruit of 51%, and 29%, respectively, compared to control treatment at in-vivo assay. This study showed the potential use of CS-TPP as a coating application for anthracnose disease management on storage chili, however further study such as viability and longevity of formula need to be done.

IntroductionCitrus hybrids with Poncirus trifoliata L. Raf. introgression have gained interest due to their tolerance to Huanglongbing (HLB), a devastating disease for Florida citrus agriculture. While these hybrids inherit disease tolerance from P. trifoliata, they sometimes also suffer from undesirable off-flavors.MethodsA selection of thirteen genotypes were harvested over the 2020-2021 and 2021-2022 seasons. Their juices were evaluated by a trained sensory panel and were comprehensively analyzed for their chemical makeup, including soluble solids content (SSC), titratable acidity (TA), volatiles, flavonoids and limonoids.Results &amp; discussionOverall, along with the commercial orange cultivars ‘Valencia’ and ‘Hamlin’, the HLB-tolerant Poncirus hybrid ‘US SunDragon,’ and the mandarin hybrids Sugar Belle®, FF-5-51-2, and ‘US Superna’ had positive citrus flavor quality. Esters, some sesquiterpenes, along with flavonoids, eriocitrin and quercetin-3-(3R-glucosylrutinoside), were positively correlated with orange flavor while β-ionone and eucalyptol were highly abundant in the mandarins. The flavonoid linarin, was more abundant in Poncirus hybrids with off-flavors than in the Poncirus hybrid ‘US SunDragon’, having high orange flavor. Two mandarin hybrids, FF-5-6-36 and FTP-6-32-67, were not bitter at harvest, but the juice exhibited delayed bitterness after storage at -20°C, which was associated with significant increases of limonin, nomilin, naringenin, and prunin. Interestingly, during freezer storage, a newly identified flavonoid in citrus, tricin-C-hexoside, increased dramatically across all of the genotypes. The identification of disease-tolerant hybrids with satisfactory flavor quality at juicing as well as after storage where delayed bitterness may develop, has great significance for future breeding efforts for fresh fruit or for use in stand-alone juice/juice blends.