Extract: Carnitine metabolism is reviewed in lipid storage myopathies, diabetes, vomiting sickness of Jamaica, malnutrition, hyperthyroidism, Duchenne dystrophy, and a few other disease states. Cases establish that for some patients, carnitine cannot be synthesized or utilized properly in the body. Carnitine metabolism should be evaluated in patients with malnutrition, especially where diets are predominantly of plant food sources.

The cellular content of total and individual phospholipids and gangliosides was measured in fibroblasts cultured from four normal subjects, three patients with lysosomal lipid storage diseases, and two subjects with homozygous familial hypercholesterolemia. Measurements were made on cells grown in medium containing fetal calf serum under conditions in which normal cells derive cholesterol for cell growth from low density lipoprotein present in the fetal calf serum, whereas familial hypercholesterolemia homozygote cells, which lack cell surface low density lipoprotein receptors, derive cholesterol from endogenous synthesis. No difference was observed in the cellular content of total or individual phospholipids and gangliosides in the normal and familial hypercholesterolemia homozygote cells. In contrast, cells from a patient with Niemann-Pick disease and a patient with Sandhoff disease showed elevations in the content of sphingomyelin and complex gangliosides, respectively.

Mannosidosis of Angus calves is an inherited lethal disease associated with a deficiency of the lysosomal enzyme α-mannosidase. It is characterised by vacuolation of neurons, macrophages, fixed reticuloendothelial cells, exocrine epithelial cells and to a lesser extent a wide variety of other cells. Vacuoles are shown to be membrane-bound vesicles and are considered to be secondary lysosomes in which are stored mannose-containing oligosaccharides that result from incomplete degradation of glycoproteins. Such glycoproteins may enter the lysosomal system by heterophagy, autophagy or crinophagy. The presence in the pancreas of zymogen granules in storage vesicles indicates that incomplete degradation of secretory granules might help explain the widespread vacuolation of other exocrine cells. It is suggested that such granules may enter the lysosomal system by crinophagy.

The articles have been scanned in colour with a HP Scanjet 5590; 300dpi. Adobe Acrobat XI Pro was used to OCR the text and also for the merging and conversion to the final presentation PDF-format. | The clinical symptoms, gross and histopathological, as well as the ultrastructural appearance of a condition in cross-bred Aberdeen Angus calves resembling the inherited lysosomal storage disease, α-mannosidosis, are reported. The neurones and perithelial cells in the brain and the reticuloendothelial cells in the lymph nodes and spleen were extensively vacuolated. The vacuoles were filled with a sparse, flocculated to granular material within which membranous structures were frequently seen. No specific substance could be identified within the vacuoles, either histochemically or ultrastructurally. Besides the vacuolation, cystic tubular structures were seen in the kidneys. The lining epithelial cells of the thyroid follicles were vacuolated while some of the follicles contained no colloid.

Botrytis allii is one of several Botrytis species causing diseases of Allium species. It causes the disease known as grey-mould neck rot which is one of the major bulb destroying diseases of the onion crop. It has caused significant losses average 10 per cent in most seasons but under conditions favourable to disease development, bulb losses of 50 per cent or more have been reported by growers. (Maude and Presly, 1977a). Tichelarr (1967) reported similar losses in Holland with averages of 10 to 20 per cent and up to 60 per cent loss of bulbs occurring. Losses caused by B. allii have also been reported in other countries including America and New Zealand. Two other related species may incite a neck rot of onion bulbs and Walker (1926) gave them the distinctive names of mycelial neck rot (B. byssoidea) and small sclerotial neck rot (B. squamosal) in order to differentiate them from grey-mould neck rot (B. allii) . Grey-mould neck rot is the major form of the disease on mature onion bulbs in New Zealadn (Brien et al., 1959) and overseas. Thus neck rot as caused by B. allii has been considered primarily to be a disease of mature bulbs which becomes evident in store, although it has been reported to cause some seedling mortality and some reported to cause some seedling mortality and some reduction of seed set in infected seed crops (Ellerbrock and Lorbeer, 1977). However, losses of bulbs in store are of primary significance because of the high cost of inputs which go into producing and storing each bulb. New Zealand exports mature bulbs and as infected bulbs are not easily identified at the time of harvest, a considerable reduction in the quality of the sample may occur as the disease progresses during transport and storage. Not only will there be a direct loss of bulbs, but the sample may be re-priced because moisture release from rotting bulbs may cause sprouting and a reduction in quality of the sample. Control of the disease has, in the past been difficult and expensive. Prior to 1970, control largely involved the manipulation of post-harvest conditions so as to minimise fungal development in store. However, in 1970, the significance of the fact that the fungus may be seed-borne was realised and the use of benomyl seed-treatments considerably reduced neck rot incidence in store (Maude and Presly, 1977b). The experiments described in this dissertation were designed to investigate the following: (1) The growth of Botrytis spp. In culture (2) The incidence of B. allii in onion seed-lines produced in New Zealand (3) The transmission of B. allii from seed to seedling (4) The testing of a range of selected fungicides for their potential in controlling B. allii.

Atlantic is a medium late maturing var. which has been shown to have the following characteristics: tolerance to scab and Verticillium wilt; high yields of attractive tubers; resistance to the bacterial disease pink eye; high solids content; excellent chip and French fry quality and acceptable fresh market quality. It is low in total glycoalkaloids. It was evaluated for yield, sp. gr., and chipping quality from 1973 through 1975 in replicated trials with locally adapted var. Compared with locally adapted var. grown in Florida, Virginia, New Jersey and Maine, Atlantic excelled in TS content. It contained 24.4% more TS than Sebago in Florida, 14.8% more than Pungo in Virginia, 14.9% more than Superior in New Jersey and 18.1% more than Superior and Katahdin in Maine. Mean chip colour scores were better for Atlantic than for locally adapted var. grown in Florida, New Jersey, and Maine. It produced excellent chips when either processed within 2 wk of harvest and then held at ambient air temp. for 6 wk or held in 4°C temp. storage and then reconditioned at 21°C for 14 days. The baking quality of Atlantic was rated acceptable.