Vacuolation abnormal

Glycogenosis type IV (branching enzyme deficiency) results in the formation of a variant of glycogen, characterized by abnormally long inner and outer glucosyl chains and fewer branch points than normal. The abnormal variant is stored in sufficient amounts to cause some vacuolation. The clinical manifestations of this... 

The major mineralocorticoid, aldosterone, is secreted by cells of the zona glomerulosa. Primary hyperaldosteronism (Conn s syndrome) is associated with potassium depletion which is, in mm, responsible for the observed neuromuscular abnormalities seen in the disorder. These are similar to those seen in hypokalemic periodic paralysis (PP), with episodic and severe exacerbations of fixed muscle weakness. Muscle biopsy shows occasional muscle necrosis and vacuoles often these feamres are accompanied by mbular aggregates as in hypokalemic PP. All these changes can be attributed to the hypokalemia and not to excess aldosterone production per se. 

In mouse glioma cells, many kinds of changes in cell organelles after exposure to 5 x 10 5 M mercuric chloride in vitro could be found [ 144]. Nuclei showed pyknotic and irregular shapes mitochondria lost their normal cristae and abnormal electron-dense areas were present within the matrix polyribosomes were dispersed. Numerous electron-dense granules and vacuolations were seen in the cytoplasm, especially around the Golgi region,... 

The lysosomal disorder SASD is characterized by accumulation of the free acid monosaccharide sialic acid in the lysosomal compartment of the cell. Diagnosis is based on the demonstration of abnormal excretion of free, not OGS-bound sialic acid in urine, coupled with accumulation of free sialic acid in cultured fibroblasts, and on microscopic evidence of vacuoles (increased and swollen lysosomes filled with light electron-lucent material in skin biopsy and peripheral blood lymphocytes). The inheritance is autosomal recessive. There are different clinical forms of this disorder an adult form, called Salla disease (SD) or Finnish sialuria (OMIM 604369) infantile SASD (ISSD OMIM 269920) and an intermediate form, severe Salla disease [3,16]. 

Excessive glycogen concentrations found in abnormal vacuoles in the cytosol... 

Results from this study showed that SOD activity was not affected by acute ADR treatment. A second finding was that acute ADR toxicity did not promote cardiac lipid peroxidation. However, it was observed that mitochondrial lipid peroxidation was highest in mice fed diets low in both antioxidants. Ultrastructural examination revealed mitochondrial abnormalities in cardiac tissue from ADR-treated animals (Figures 3 and 4). There were large vacuoles within the mitochondria and condensation of the inner and outer membranes of the mitochondria. The ultrastructural effects of ADR treatment were most severe in the low E, Mn-deficient mice. It is reasonable to suggest that a higher than normal level of lipid peroxidation may predispose the animal to tissue damage from ADR. Consistent with this concept, Meyers et al. (34) have reported that pretreatment with supplemental vitamin E can reduce the toxicity of ADR in mice. 

Rohovsky, Newberne and Gibson17 reported the appearance of vacuolation and granulation of peripheral leucocytes in the mouse, rat, dog and monkey. A concomitant alteration in the reticuloendothelial system also occurred which consisted of intracytoplasmic accumulation of ovoid structures with vacuolated centers in Kupffer cells of the liver and mixed macrophages of the spleen and lymph nodes. Smears of bone marrow revealed abnormal granules in myeloid cells as primitive as neutrophilic myelocytes, but the myeloid erythoid ratio was not altered. The post-treatment regression of these effects was species dependent. 

Several NMDA receptor antagonists have been synthesized and tested in stroke models, epilepsy models, and in clinical trials. Many of the antagonists are limited by side effects such as hemodynamic abnormalities, hypotension, neuronal vacuolation, memory disturbances, cognitive disturbances, motor dysfunction, seizures, hallucinations, unpleasant dreams, psychotomimetic episodes, and other effects. This has made the search difficult for NMDA receptor antagonists that can be used as neu-roprotective agents. 

Acute renal toxicity may occur within days of initiating therapy. Serum creatinine concentration rises and creatinine clearance decreases. Hypertension, hyperkalemia, sodium avidity, and hypomagnesemia may occur. No urine sediment abnormalities are seen. Urinary enzyme excretions increase, but are not reliable indicators of toxicity. Renal biopsy reveals thickening of arterioles, mild focal glomerular sclerosis, proximal tubular epithelial cell vacuolization and atrophy, and interstitial fibrosis. Biopsy is useful to distinguish acute cyclosporine nephrotoxicity from renal allograft rejection, the latter being evidenced by cellular infiltration. ... 

Atypical and classical forms of scrapie differ in a number of important respects. Sheep with atypical scrapie are invariably older, and cases are frequently only found in single animals in an infected flock [40]. Unlike the pathology of classical scrapie, there is little or no neuronal vacuolation or immunohistochemically-detect-able PrPSc in the obex region of the brain with atypical scrapie cases [37]. Abnormal PrP in atypical cases has been shown to be more susceptible to enzymatic digestion than in classical scrapie [41]. Also, characterization of protease-resistant PrPSc by Western blot shows a unique biochemical signature that is shared by all atypical scrapie cases, most prominently, a characteristic band of apparent low molecular weight 12 kDa that is not seen in analyses of classical scrapie cases [37]. Finally, atypical scrapie occurs in sheep with PRNP genotypes usually associated with resistance to classical scrapie, particularly with the AHQ allele. Sheep with atypical scrapie are more likely to express phenylalanine (F) than leucine (L) at codon 141 [40]. 

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