Cell death has been traditionally classified in apoptosis and necrosis. Apoptosis, known as programmed cell death, is an active form of cell death mechanism that is tightly regulated by multiple cellular signaling pathways and requires ATP for its appropriate process. Apoptotic death plays essential roles for successful development and maintenance of normal cellular homeostasis in mammalian. In contrast to apoptosis, necrosis is classically considered as a passive cell death process that occurs rather by accident in disastrous conditions, is not required for energy and eventually induces inflammation. Regardless of different characteristics between apoptosis and necrosis, it has been well defined that both are responsible for a wide range of human diseases. Glycogen storage disease type I (GSD-I) is a kind of human genetic disorders and is caused by the deficiency of a microsomal protein, glucose-6-phosphatase-α (G6Pase-α) or glucose-6-phosphate transporter (G6PT) responsible for glucose homeostasis, leading to GSD-Ia or GSD-Ib, respectively. This review summarizes cell deaths in GSD-I and mostly focuses on current knowledge of the neutrophil apoptosis in GSD-Ib based upon ER stress and redox signaling.

A case of lysosomal storage disease has been reported in a calf of Japanese Black cattle. Lysosomal storage diseases are hereditary diseases caused by deficiency of lysosomal hydrolases. The clinical and pathological features and accumulated substrates of the affected animal indicated a possibility of sialidosis or galactosialidosis caused by deficiency of neuraminidase (NEU1) or protective protein for β-galactosidase (PPGB). In the present study, we investigated nucleotide sequences of the genes encoding these two proteins to evaluate whether mutation of these genes is involved in this disease. We determined cattle genomic sequences of these two genes by using bovine EST sequences and the nucleotide sequences of all exons of these genes were compared between affected and normal animals. The results showed several nucleotide substitutions, but none of them was a functional mutation or specific to the affected animal. Furthermore, genotyping of the microsatellite markers in the vicinity of these two genes revealed no homozygosity of the chromosomal regions including these genes in the affected animal. These findings indicated that neither NEU1 nor PPGB gene is responsible for the lysosomal storage disease of Japanese Black cattle and therefore the disease is neither sialidosis nor galactosialidosis.

Clinical and pathologic findings for the spontaneous poisoning by Sida carpinifolia in cattle are described in this study. A survey on field cases of S. carpinifolia in cattle was carried out on farms of Alto Vale do Itajai, State of Santa Catarina, southern Brazil. Sixteen affected animals were clinically evaluated and 9 were subjected to postmortem examination. The main clinical signs consisted of marching gait, alert gaze, head tremors, and poor growth. Histologic and ultrastructural lesions consisted of vacuolization and distension of neuronal perikarya, mainly from Purkinje cells, and of the cytoplasm of acinar pancreatic and thyroid follicular cells. Clinical signs and lesions varied from mild to severe. Improvement of the clinical signs was observed in cattle after a period of up to 90 days without consuming the plant; however, residual lesions, mainly characterized by axonal spheroids and absence of Purkinje neurons in some areas of the cerebellum, were observed in these cases. It is concluded that the natural chronic consumption of S. carpinifolia was the etiologic cause of storage disease in cattle in this study.

Five fungicides were each applied on sugar beet (Beta vulgaris) three times at about 14-day intervals beginning in July in 2005 through 2008. No foliar disease occurred in the nontreated control or any fungicide treatment. There were no significant differences in root yield, recoverable sucrose, or sucrose concentration among treatments. In 2007 and 2008, postharvest storage respiration rates were determined after 30 and 90 days in storage. There was no significant difference in respiration rates among treatments. There was no apparent benefit in applying these fungicides in seasons when no foliar disease developed.

Combining different control methods can improve control efficacy, increase the spectrum of controlled pathogens and reduce the possibility of resistance development. To be successful, however, the different methods need to be compatible: the first treatment should not have any deleterious effect on the succeeding one; preferably, it should contribute to its efficacy. In the last few years, carrot growers in Israel have begun to brush carrots before storage to remove the outer peel of the root. In the present study we show that this practice enhances the appearance of Black Root Rot during storage, a postharvest disease caused by the fungus Thielaviopsis basicola. The chemical fungicide iprodione is usually applied before storage to reduce the development of postharvest diseases. We evaluated the efficacy of combining physical, low-residue chemical and biological control agents as an alternative to the conventional chemical control approach. A technology for the precise application of steam and combined application with stabilized hydrogen peroxide (Tsunami® 100) or a yeast commercial product (Shemer[trade mark sign]) were tested. Used alone, both the steam and Tsunami were highly effective at reducing disease decay but were phytotoxic to the roots. Application of combined treatments of sublethal steam followed by a sublethal dosage of Tsunami or Shemer improved efficacy and disease control by 80 and 86%, respectively. These combinations showed a synergistic effect as compared to each of the treatments alone. The same pattern, effecting up to 54% disease control, was observed with the non-compatible combination of applying Tsunami first, washing it off with water and then applying Shemer. Thus disease-control agents can potentially be used for a short period, then washed off, if needed, and efficiently followed by application of a biological control agent. The biological pathway and mode of action are still under investigation but to the best of our knowledge, this is the first study to mathematically demonstrate synergistic effects of sublethal treatments applied sequentially to control postharvest disease as a potential method to reduce the use of chemicals in fruit and vegetables.

The fungus Botrytis cinerea is the major cause of decay in table grapes during storage, and the severity of decay depends in part on contamination with the fungus before storage. The current SO₂ technology to prevent decay is robust and independent of the level of contamination by B. cinerea. The introduction of alternative technologies may however require implementation of means which are proportional to the level of contamination. The objectives of this study were to test the feasibility of quantifying B. cinerea in artificially inoculated grapes and to monitor the progress of disease during storage. Two methods were compared for detection of B. cinerea in grapes; an antibody kit specific for B. cinerea, and quantitative PCR using fungal specific primers. Antibodies for fast detection of B. cinerea yielded positive results only in the later stages of decay development. In contrast, the quantitative PCR demonstrated positive identification of the fungus at all storage time points, and found increasing amounts of the fungus during storage.

Niemann-Pick type C (NPC) is a fatal autosomal recessive lysosomal storage disease clinically characterized by neurodegeneration and liver disease. Heterogeneous mutations in the NPC1 and NPC2 genes cause impaired egress of free cholesterol from lysosomes, leading to accumulation of cholesterol and glycosphingolipids. Key features of NPC liver disease include hepatic apoptosis, inflammation, and fibrosis. It is unclear what signaling events regulate these disease processes in NPC. We hypothesize that tumor necrosis factor α (TNF-α), which is involved in both proinflammatory and apoptotic signaling cascades, is a key mediator of inflammation, apoptosis, and fibrosis in NPC liver disease. In this study, we evaluated the role of TNF-α signaling in NPC liver disease by utilizing NPC1-specific antisense oligonucleotides to knock down NPC1 expression in control and TNF-α knockout mice. In the absence of TNF-α, NPC1 knockdown produced liver disease with significantly less inflammation, apoptosis, and fibrosis.

The production of free radicals in cells and body tissues has been linked to many aging-related disease. Interest in nutraceutical content of foods has intensified these days because of their health benefits as dietary antioxidants. Anthocyanin which are responsible for the red, purple and blue colors of many fruits, vegetables, flowers and cereal grains, are known to reduce the coagulation of blood platelets, inhibit formation of blood clots involved in stroke, vascular disease and heart attack, and promote higher levels of 'good' cholesterol. These compounds are present substantially in pigmented rice making it an enriched form of rice for taste and health benefits. PhilRice has successfully produced anthocyanin-rich tapuy from pigmented rice with good acceptability and high amount of anthocyanin. In order to investigate the storage stability of pigmented rice wine at room (27-30 deg C), cold (16-19 deg C), and refrigerated (4-7 deg C) conditions, shelf life evaluation was conducted. The physicochemical properties, anthocyanin content, microbial load, and sensory characteristics were monitored over six months. The color of wine slightly darkened after the storage period for all samples. No major changes on acidity and sweetness in the wine samples for all storage conditions while the anthocyanin content decreased as the storage time and temperature increased. No coliforms, molds or yeasts were detected in all samples while acceptable bacterial count was found in cold and refrigerated samples. Turbidity of wine was noticed with increasing time and decreasing temperature, with refrigerated sample being the most turbid. It was also observed that the overall acceptability of the samples decreased as the storage time and temperature increased. Further evaluation will be conducted to determine the optimum shelf-life of anthocyanin-rich red wine.

Sandhoff disease (SD) is a glycosphingolipid (GSL) storage disease that arises from an autosomal recessive mutation in the gene for the β-subunit of β-Hexosaminidase A (Hexb gene), which catabolizes ganglioside GM2 within lysosomes. Accumulation of GM2 and asialo-GM2 (GA2) occurs primarily in the CNS, leading to neurodegeneration and brain dysfunction. We analyzed the total lipids in the brains of SD mice, cats, and humans. GM2 and GA2 were mostly undetectable in the normal mouse, cat, and human brain. The lipid abnormalities in the SD cat brain were generally intermediate to those observed in the SD mouse and the SD human brains. GM2 comprised 38, 67, and 87% of the total brain ganglioside distribution in the SD mice, cats, and humans, respectively. The ratio of GA2-GM2 was 0.93, 0.13, and 0.27 in the SD mice, cats, and humans, respectively, suggesting that the relative storage of GA2 is greater in the SD mouse than in the SD cat or human. Finally, the myelin-enriched lipids, cerebrosides and sulfatides, were significantly lower in the SD brains than in the control brains. This study is the first comparative analysis of brain lipids in mice, cats, and humans with SD and will be important for designing therapies for Sandhoff disease patients.

The effect of coating with irradiated Crab and Shrimp chitosan (CHIirr, Mv = 5.14 × 104) and un-irradiated Crab chitosan (CHIun, Mv = 2.61 × 105) on postharvest preservation of mango (Mangifera indica L.) fruit was studied. Irradiation at 100 kGy and 200 kGy of both Crab chitosan and Shrimp chitosan were used and the fruits were stored at 15 degree C plus minus 1 degree C and 85% relative humidity for 6 weeks. The effect of various chitosan coatings on fruit ripening behaviour, biochemical and organoleptic characteristics were evaluated during storage. The incidence of disease attack was also observed. The overall results showed the superiority of irradiated Crab chitosan (200 kGy) in extending the shelf-life of mango fruit as compared to control. The irradiated Crab chitosan (200 kGy) treated fruits also maintained their eating quality up to 4 weeks of storage. Only 6% disease incidence was observed in fruits coated with irradiated Crab chitosan (200 kGy) as compared to c