We report a family of French Canadian and Dutch ancestry with hereditary ferritinopathy (neuroferritinopathy) and a novel mutation (C insertion at nt646-647 in exon 4) in the ferritin light chain ...gene, resulting in a longer than normal protein. Our failure to immunostain most of the abnormal ferritin deposits in the proband with a conformation-dependent monoclonal antibody to ferritin light chain supported a previously postulated conformational change of ferritin light chain in this disease. The posterior putamen and cerebellum were the primary pathologic loci in our proband, but asymptomatic hepatocytic intranuclear accumulations of iron and ferritin also were present. Both neurons and glia displayed highly distinctive, if not pathognomonic, swollen to vacuolated nuclei containing ferritin and iron. Hyaline deposits, again staining for both ferritin and iron, were additional morphologic features that may be unique to the ferritinopathies. The iron, at least in putamen where there was a nearly 40-fold increase, appeared to be both in the ferrous (Fe) and ferric (Fe) form; it was the most likely cause of the observed neuronal and glial apoptosis. We found morphologic evidence of both lipid peroxidation and abnormal nitration of proteins in putaminal neurons and glia, confirming the expected oxidative stress due to this excessive iron. Biochemical and immunohistochemical abnormalities in mitochondria also were demonstrated, probably due to an imbalance in iron homeostasis that had a deleterious effect on the respiratory chain.
As biotrophs, insidious fungal infections of postharvest pathogens remain quiescent during fruit growth while at a particular phase during fruit ripening and senescence the pathogens transform to ...necrotrophs causing typical decay symptoms. Exposure of unripe hosts to pathogens (hemi-biotroph or necrotrophs), initiates defensive signal-transduction cascades that limit fungal growth and development. Exposure to the same pathogens during ripening and storage activates a substantially different signalling cascade which facilitates fungal colonization. This chapter will focus on modulation of postharvest host-pathogen interactions by pH and the consequences of these changes. Host pH can be raised or lowered in response to host signals, including alkalization by ammonification of the host tissue as observed in Colletotrichum and Alternaria, or acidification by secretion of organic acids as observed in Penicillium, Botrytis and Sclerotinia. These changes sensitize the host and activate transcription and secretion of fungal hydrolases that promote maceration of the host tissue. Several particular examples of coordinated responses which follow this scheme are described.
The commonly used method of static radionuclide ventriculography with a cardiosynchronizer does not permit obtaining a curve of ventricular function over a complete cardiac cycle, especially using ...computers with a small memory volume. Besides, the final segment of a curve is usually insignificant as a result of variations in a duration of the R-R interval. A method of 2-stage recording of radionuclide ventriculography has been proposed, the second stage being shifted to a certain time interval, i. e. a delay line is introduced between a synchronizing device and computer. Two methods of delay--in the software and hardware--are proposed, the latter being more preferable as it makes unnecessary extra technical devices. Particular attention is drawn to a choice of delay time depending on a computer memory volume: in a small memory volume delay time must be approximately one-third of the patient's R-R interval.
Based on the regularity of decreasing the blood volumes from the internal edge of the lung to its periphery the authors proposed a method of gradient background correction in inhalation scintigraphy ...of the lungs with 133Xe. The background is detached on each image line separately for the right and left lungs. Software was developed for the implementation of this technique of background correction for any computer with a FORTRAN-IV translator.
