A glycogen storage disease affecting primarily the skeletal muscle and, to a lesser degree, the cardiac muscle, spinal cord, and brain was diagnosed in a 10-year-old neutered Abyssinian cat with a 4-year history of paresis progressing to acute paralysis. Microscopically, these tissues contained inclusions that were pale basophilic in hematoxylin and eosin-stained slides, diastase resistant, periodic acid-Schiff positive, and blue-to-almost black with iodine stain. By transmission electron microscopy, the inclusions consisted of cytosolic, usually sharply demarcated, nonmembrane-bound deposits of finely granular and filamentous material. on the basis of the structural and histochemical staining characteristics, the inclusions were believed to be aggregates of abnormally stored, unbranched glycogen. A defect in glucose metabolism is suspected to be the underlying pathologic process, but an exact cause remains elusive.

Onions of the variety Granex 33 were placed in low humidity, warm air storage for a minimum of 15 weeks and then examined for weight loss, pungency, soluble solids, and disease presence. They were compared with onions held in cold and CA storage. Temperature and relative humidity of the air in the low humidity, warm air storage established levels as generated by a heat-pump-dehumidifier with means 30 degrees C to 34 degrees C and 29% to 36% R.H. over years, there being a 0.2 degrees C to 0.6 degrees C and 0.1% to 0.6% R.H. differential between intake and exhaust conditions. Low humidity, warm air storage proved suitable for mid-term storage of healthy onions or those onions with shallow surface diseases whose disease development could be halted. Onions containing hidden internal diseases were exasperated by the warm circulating air, causing further disease development.

Fusarium basal rot (FBR), caused by Fusarium oxysporum Schlechtend.:Fr. f. sp. cepae (H.N. Hans.) W.C. Snyder & H.N. Hans, is a soilborne fungal disease that affects bulb onions (Allium cepa L.) worldwide. Short-day onion cultivars that are resistant to FBR are lacking. The goal of this project was to screen fall-sown onion germplasm for FBR resistance using a mature bulb field screening at harvest and after 4 weeks in storage. The project was conducted for 2 years, and in each year, 26 fall-sown onion lines were grown in a field known to produce a high incidence of fusarium-basal-rot-infected bulbs. When all the bulbs in a plot were mature, the basal plates of 20 bulbs were cut transversely and each plate was rated for disease severity on a scale of one (no diseased tissue) to nine (70% or more diseased). Bulbs were stored and rerated at 2 and 4 weeks after harvest. Disease severity and incidence were higher in the first year than in the second year. Both severity and incidence increased as bulbs were stored for 4 weeks. NMSU 00-25 exhibited the lowest disease severity and incidence in both years at harvest time and after storage. 'Buffalo' and 'Cardinal' exhibited the highest severities and incidences across both years and at harvest time and after storage. Other entries exhibited high or low disease severity and incidence but not consistently across years and between harvest time and after storage. In the development of FBR resistant cultivars, breeding lines should be evaluated over multiple years and bulbs should be stored for 4 weeks before being screened.

Gaucher disease is a common lysosomal storage disease caused by a defect of acid β-glucosidase (GCase). The optimal in vitro hydrolase activity of GCase requires saposin C, an activator protein that derives from a precursor, prosaposin. To develop additional models of Gaucher disease and to test in vivo effects of saposin deficiencies, mice expressing low levels (4–45% of wild type) of prosaposin and saposins (PS-NA) were backcrossed into mice with specific point mutations (V394L/V394L or D409H/D409H) of GCase. The resultant mice were designated 4L/PS-NA and 9H/PS-NA, respectively. In contrast to PS-NA mice, the 4L/PS-NA and 9H/PS-NA mice displayed large numbers of engorged macrophages and nearly exclusive glucosylceramide (GC) accumulation in the liver, lung, spleen, thymus, and brain. Electron microscopy of the storage cells showed the characteristic tubular storage material of Gaucher cells. Compared with V394L/V394L mice, 4L/PS-NA mice that expressed 4–6% of wild-type prosaposin levels had ∼25–75% decreases in GCase activity and protein in liver, spleen, and fibroblasts.These results imply that reduced saposin levels increased the instability of V394L or D409H GCases and that these additional decreases led to large accumulations of GC in all tissues. These models mimic a more severe Gaucher disease phenotype and could be useful for therapeutic intervention studies.

The cold storage of Mairac® apples was tested from 2002 to 2004. The objective was to compare the cold storage in controlled atmosphere CA and ULO (Ultra Low Oxygen) and to determine the optimal harvest period. The cold storage of Mairac® apples in ULO gave the best results. Quality parameters such as firmness and malic acid content of the fruits were better and the propagation and intensity of core browning was reduced. The cold storage of Mairac® apples in ULO conditions allows maintaining the quality and reducing the brown heart disease till the month of May.

Specific and sensitive quantitative diagnostics, based on real-time (TaqMan) polymerase chain reaction (PCR) and PCR enzyme-linked immunosorbent assay, were developed to detect dry-rot-causing Fusarium spp. (F. avenaceum, F. coeruleum, F. culmorum, and F. sulphureum). Each assay detected Fusarium spp. on potato seed stocks with equal efficiency. Four potato stocks, sampled over two seed generations from Scottish stores, were contaminated with F. avenaceum, F. sulphureum, F. culmorum, F. coeruleum or a combination of species, and there was a general trend towards increased Fusarium spp. contamination in the second generation of seed sampled. F. sulphureum and F. coeruleum caused significantly (P < 0.05) more disease in storage than the other species when disease-free tubers of potato cvs. Spunta and Morene were inoculated at a range of inoculum concentrations (0, 10(4), 10(5), and 10(6) conidia/ml). Increased DNA levels were correlated with increased disease severity between 8 and 12 weeks of storage. The threshold inoculum levels resulting in significant disease development on both cultivars were estimated to be 10(4) conidia/ml for F. sulphureum and 10(5) conidia/ml for F. coeruleum. To study the effect of soil infestation and harvest date on disease incidence, seed tubers of cvs. Morene and Spunta were planted in a field plot artificially infested with the four Fusarium spp. F. culmorum and F. sulphureum were detected in soil taken from these plots at harvest, and F. sulphureum DNA levels increased significantly (P < 0.05) at the final harvest. All four Fusarium spp. were detected in progeny tubers. There was a trend toward higher levels of F. culmorum detected in progeny tubers at the earliest harvest date, and higher levels of F. sulphureum at the final harvest. The use of diagnostic assays to detect fungal storage rot pathogens and implications for disease control strategies are discussed.

The epidemiology of sclerotinia rot of carrot was investigated on carrot 'Cellobunch' during 1999 and 2000 in Ontario. Apothecia were first detected in the crop in early August to mid-September, after the carrot canopy closed and after 7-11 days with soil matric potentials between -0.1 and -0.4 bars and soil temperatures between 14 and 23 degrees C. Ascospores were first detected in mid-July to mid-August, usually before apothecia were observed in the crop, and after 7-12 days with soil matric potentials between -0.1 and -0.3 bars and air temperatures between 15 and 21 degrees C. The numbers of apothecia and ascospores were positively correlated with soil matric potential. Preharvest epidemics started in mid-August to mid-September, after the closure and lodging of the canopy, after the appearance of senescing leaves on the soil and ascospores in the crop, and after rain had initiated 12-24 h per day of leaf surface wetness. Disease incidence was negatively correlated with air and soil temperatures. Postharvest epidemics in storage followed preharvest epidemics in the field, but not all preharvest epidemics resulted in disease in storage. It is suggested that severe epidemics of sclerotinia rot of carrot can occur in storage when disease in the field progresses rapidly and is associated with soil matric potentials of greater than or equal to -0.2 bars and leaf wetness of greater than or equal to 14 h per day, particularly close to harvest. The information revealed in this study contributes to the development of inoculum and disease prediction systems and improved management of sclerotinia rot of carrot.

<p>Abstract</p> <p>Background</p> <p>Cholesterol ester storage disease (CESD) is an autosomal recessive illness that results from mutations in the <it>LIPA </it>gene encoding lysosomal acid lipase. CESD patients present in childhood with hepatomegaly and dyslipidemia characterized by elevated total and low-density lipoprotein cholesterol (LDL-C), with elevated triglycerides and depressed high-density lipoprotein cholesterol (HDL-C). Usual treatment includes a low fat diet and a statin drug.</p> <p>Results</p> <p>In an 18-year old with CESD, we documented compound heterozygosity for two <it>LIPA </it>mutations: a novel frameshift nonsense mutation and a deletion of exon 8. The patient had been treated with escalating doses of lovastatin for ~80 months, with ~15% decline in mean LDL-C. The addition of ezetimibe 10 mg to lovastatin 40 mg resulted in an additional ~16% decline in mean LDL-C.</p> <p>Conclusion</p> <p>These preliminary anecdotal findings in a CESD patient with novel <it>LIPA </it>mutations support the longer term safety of statins in an adolescent patient and provide new data about the potential efficacy and tolerability of ezetimibe in this patient group.</p>

Blue mold caused by Penicillium expansum is define as major disease inflicted heavy losses to cold storage apple fruits. As a result of investigations in Fruitgrowing Institute in Plovdiv as a potential antagonist were isolated and tested 264 morphologically different microorganisms. After primary and secondary screening 10 isolated were selected. A biotest was carried out with them in cold storage. Three isolates inhibit the development of blue mold over 50%. The influence of the concentration of pathogen and isolate spores and the mechanism of antagonistic action are in process of study.

Purpose - Consumer food handling behaviour is important in preventing food borne disease and this paper proposes examining consumer behaviour and knowledge concerning food storage and disposal. Design/methodology/approach - Interviews and observations were used to investigate the storage methods and the storage durations of various categories of food products as well as the consumers' motives when they decide to throw away food products. The temperature of their refrigerators was also measured. Findings - The results demonstrate that consumers deal with meat and dairy products relatively hygienically, but the storage of products in other categories is more likely to lead to risks; especially because of high storage temperatures. The study also shows that the elderly handle food differently from younger people. Research limitations/implications - Knowledge of the recommended storage methods and the consequences of consuming out-of-date foodstuffs have not been investigated. This could be dealt with in a supplemental research. Practical implications - The elderly have a different purchasing policy. The older consumer of the future is likely to act differently to the present day elderly with respect to the disposal of food products. Originality/value - Reveals that consumer education about food safety, in particular food storage and food handling is recommended.