Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:1.3.5.1 (succinate dehydrogenase)
 8,177 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Giant osteoclasts and other cells were isolated from Pagetic bone tissue using 0.5 mM ethylene diamine tetraacetic acid on bone samples from 8 patients with Paget's disease. The cell suspension contained osteoclasts and osteoblasts as well as some mononuclear cells such as monocytes. The number of nuclei in isolated osteoclasts (33.85 +/- 20.92 nuclei/osteoclast) correlates fairly well (p less than 0.02) with the number of nuclei counted on histologic sections (15.88 +/- 11.80 nuclei/osteoclast) for samples from each patient. Enzyme histochemistry demonstrated acid phosphatase activity in isolated osteoclasts and in mononucleated cells, such as monocytes. Alkaline phosphatase was detected only in osteoblasts while succinate dehydrogenase was observed in osteoclasts, osteoblasts and monocytes. Esterases, such as nonspecific aliesterase and specific naphthol AS-D acetate esterase, were identified in osteoclasts and in macrophages. Inhibition of specific naphthol AS-D acetate esterase in osteoclasts by addition of sodium fluoride suggests that the enzyme could be of monocytic origin.
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PMID:Isolation of osteoclasts from Pagetic bone tissue: morphometry and cytochemistry on isolated cells. 283 60

Observations made during a growth and development study of the semitendinosus and trapezius muscles of 49 purebred Large White pigs between birth and 128 days of age revealed the presence of giant fibres. The occurrence, histochemical and ultrastructural properties of these giant fibres were investigated. A high proportion of the pigs (85 per cent) contained giant fibres in their muscles but these giant fibres usually represented less than 1 per cent of the total myofibre population. Giant fibres possessed enhanced adenosine triphosphatase activity and a high capacity for oxidative metabolism (indicated by succinate dehydrogenase activity) which was reflected ultrastructurally by the greatly heightened electron density of myofibrils and by an abnormally high percentage of mitochondria and lipid droplets. These deviations from normal muscle fibre composition, together with the reduced percentage volume of sarcoplasmic reticulum, were consistent with changes seen in functionally over-loaded muscle. It appears that giant fibre anomalies occur through increased activity stimulated in occasional muscle fibres, perhaps by a structural defect, such as an inadequate amount of sarcoplasmic reticulum, which causes hyper-contractile activity within the fibres and associated compensatory adaptations. Giant fibres did not appear to represent fibres undergoing degenerative changes.
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PMID:"Giant" muscle fibres in skeletal muscle of normal pigs. 294 80

The characteristic myopathic features revealed by histological observations included strong proliferation of connective and fatty tissue, perivascular infiltrations and necrosis of muscle fibers with phagocytosis to the lesser extent. In the myopathic muscle, as well as in giant fibers, histochemical techniques showed a reduction in succinate dehydrogenase and lactate dehydrogenase activity in type beta R (slow-twitch, oxidative) and alpha R (fast-twitch, oxidative and glycolytic). Magnesium-activated adenosine triphosphatase reaction ranged from diffuse to negative in beta R, alpha R and alpha W (fast-twitch, glycolytic) fiber types. Diffuse reaction for acid phosphatase and total loss of glycogen content were observed. The micrographs of the myopathic muscle indicated enlarged mitochondria with atrophy or complete destruction of cristae. Many myofibrils were hypercontracted. Giant fibers possessed mitochondria enlarged to an even greater extent and many of the myofibrils had loss of continuity, were narrow, depleted and were also hypercontracted. Significant differences between myopathic and normal groups were found in number of beta R fibers (lower in the myopathic group), number of alpha R fibers and percent of alpha R and alpha W fibers (higher in the myopathic group). Differences (P less than .01) existed between meat pH1 value in the myopathic group (mean value of 5.95) and the normal group (mean value of 6.29). Meat from the myopathic group of pigs also had a lower (P less than .01) pH24 value and reduced water-holding capacity (P less than .01) relative to the meat of the normal pigs. The lack of difference of fattening and slaughter traits between the groups suggested that the White Zlotnicka pigs is of particular value because it is possible to improve the production traits without increasing the incidence of these syndromes within the breed. Negative correlations (P less than .05) between number of giant fibers and percent of alpha W fibers, and between percent of giant fibers and percent of alpha W fibers indicate that alpha W fibers can undergo degeneration and be transformed into giant fibers. Therefore, it it suggested that giant fibers should be treated as muscular, pathological results of past stresses and not as an additional type of normal muscle cells.
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PMID:Histopathological observation of stress myopathy in M. longissimus in the pig and relationships with meat quality, fattening and slaughter traits. 362 2

Enzyme histochemical techniques were used as markers of macrophage activity and differentiation in the periodontal tissues following orthodontic tooth movement in man. The enzymes studied included lactate dehydrogenase, glucose-6-phosphate dehydrogenase, succinic dehydrogenase, acid phosphatase and its tartrate resistant isoenzyme, arylsulfatase, aminopeptidase M and prostaglandin synthetase. Chloroacetyl esterase activity was studied in order to detect possible neutrophilic degrading activity. Intense activities of arylsulfatase and prostaglandin synthetase and a moderate activity of aminopeptidase M were found in cells degrading the hyaline zone. However, no activity of tartrate resistant acid phosphatase was found in these cells. Giant cells in contact with bone surfaces adjacent to the hyaline zone exhibited an intense activity of succinic dehydrogenase, tartrate resistant acid phosphatase and aminopeptidase M. Chloroacetyl esterase activity did not change following orthodontic treatment. The results indicate that macrophages in various stages of differentiation were responsible for the degradation of the hyaline zone and alveolar bone during orthodontic tooth movement. The enzymatic differences were probably due to the influence of the immediate cellular environment. Prostaglandin synthetase activity, which may be interpreted as a sign of prostaglandin secretion, was associated with the degradation of the hyaline zone in man.
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PMID:Cellular enzyme activity associated with tissue degradation following orthodontic tooth movement in man. 657 20

The aim of this study was to review critically the diagnostic features of intestinal neuronal dysplasia type B (IND B). Over a period of 5 years colonic mucosal biopsies of 773 children with symptoms of chronic constipation were examined. Four biopsies taken 2-10 cm above the pectinate line were cut in serial sections and histochemical lactate dehydrogenase, succinate dehydrogenase, (SDH) and acetylcholinesterase (AChE) reactions performed. Presence of giant ganglia of the submucosal plexus, being characterized by more than seven nerve cells, established the diagnosis of IND B. Giant ganglia were found to be age-independent changes, while hyperplasia of the submucosal plexus, increase of AChE activity in nerve fibres of the lamina propria and low SDH activity in nerve cells proved to be age-dependent findings which disappear during the maturation of the enteric nervous system. Using these criteria IND B was diagnosed in 209 children. In 64 of these patients a combination of IND B and aganglionosis (Hirschsprung's disease) was found. IND B seems to be related to premature expression of laminin A during embryogenesis, resulting in premature nerve cell differentiation in the myenteric and submucosal plexus, which in turn blocks neuroblast colonization of the rectum. IND B, hypoganglionosis and aganglionosis, which are often combined, may therefore be considered to be different manifestations of the same developmental abnormality.
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PMID:Histopathological criteria for intestinal neuronal dysplasia of the submucosal plexus (type B) 754 72

Mouse renal cell tumors (RCT) were induced in male CBA male mice by 5 subcutaneous injections of 8 mg 1,2-dimethylhydrazine (DMH) per kg body weight once a week. After a lag period of two years the kidneys were removed, and serial cryostat sections of the kidneys were histochemically analyzed for the following parameters: Glycogen content, basophilia, and activities of glycogen synthase (SYN), glycogen phosphorylase (PHO), glucose-6-phosphatase (G6Pase), glucose-6-phosphate dehydrogenase (G6PDH), hexokinase (HK), pyruvate kinase (PK), lactate dehydrogenase (LDH), malic enzyme (ME), succinate dehydrogenase (SDH), alkaline phosphatase (ALPase) and glutamyl-transpeptidase (GGT). RCT displayed the same histochemical profile irrespective of their size and growth pattern. In comparison with normal kidney epithelium, the neoplastic cells exhibited elevated activities of enzymes for glycolysis (HK, PK LDH) and the pentose phosphate pathway (G6PDH) while negative G6Pase and low SDH activity were observed in these cells. The majority of RCT showed high PHO activity and weak staining for SYN. Activities of ALPase and GGT were negative in most of the RCT. Giant cells were detected in some large RCT. Higher activities of glycolytic and mitochondrial enzymes and G6PDH were found in giant cells compared with other tumor cells. Tubular preneoplastic lesions were similar to neoplastic lesions in morphological and histochemical characteristics. The present study revealed that a markedly elevated capacity for glycolysis and the pentose phosphate pathway occurred in renal cell tumors in mice. A similar histochemical pattern in the few preneoplastic tubular lesions observed suggests that these metabolic aberrations emerge early in carcinogenesis, but studies on earlier stages of renal carcinogenesis are needed to substantiate this assumption.
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PMID:[Enzymic spectrum of preneoplastic and neoplastic changes induced by 1,2-dimethylhydrazine in mouse kidneys]. 874 89

This study examined the frequency, morphological and immunohistochemical characteristics of the giant fibres in the longissimus muscle of local Krsko polje pigs with different Ryr1 genotypes. Giant fibres were round-shaped and had significantly increased cross-sectional area compared with normal muscle fibres. Only fast-twitch glycolytic fibres were affected, usually showing enhanced succinate dehydrogenase activity. On the ultrastructural level, the dilation of the sarcoplasmic reticulum, swelling of mitochondria and destruction of myofilaments was observed. The incidence of giant fibres was the highest in Ryr1 dimutant pigs (Ryr1 nn), which also exhibited lower muscle pH1 than heterozygous (Ryr1 Nn) or pigs with the wild Ryr1 gene (Ryr1 NN). However, the giant fibres were also present in pigs free of Ryr1 gene mutation. Our results suggest that the giant fibre syndrome depends mostly upon the rate and intensity of early post-mortem glycolysis, which results in acidity of muscle tissue. We suppose that the giant fibre formation is a result of excessive intracellular lactate accumulation in some fast-twitch glycolytic fibres. This process could also explain the ultrastructural alterations and the consequent changes in the oxidative enzymes and myofibrillar ATPase staining pattern observed in our and some previous studies.
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PMID:Giant muscle fibres in pigs with different Ryr1 genotype. 1269 57

Size is a critical property of a cell, but how it is determined is still not well understood. The sepal epidermis of Arabidopsis (Arabidopsis thaliana) contains cells with a diversity of sizes ranging from giant cells to small cells. Giant cells have undergone endoreduplication, a specialized cell cycle in which cells replicate their DNA but fail to divide, becoming polyploid and enlarged. Through forward genetics, we have identified a new mutant with ectopic giant cells covering the sepal epidermis. Surprisingly, the mutated gene, SEC24A, encodes a coat protein complex II vesicle coat subunit involved in endoplasmic reticulum-to-Golgi trafficking in the early secretory pathway. We show that the ectopic giant cells of sec24a-2 are highly endoreduplicated and that their formation requires the activity of giant cell pathway genes LOSS OF GIANT CELLS FROM ORGANS, DEFECTIVE KERNEL1, and Arabidopsis CRINKLY4. In contrast to other trafficking mutants, cytokinesis appears to occur normally in sec24a-2. Our study reveals an unexpected yet specific role of SEC24A in endoreduplication and cell size patterning in the Arabidopsis sepal.
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PMID:Endomembrane trafficking protein SEC24A regulates cell size patterning in Arabidopsis. 2531 6

Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Over the past two decades, our increasing understanding of the molecular and genetic etiologies of pituitary gigantism and acromegaly yielded several genetic causes, including multiple endocrine neoplasia type 1 and 4, McCune-Albright syndrome, Carney complex, familial isolated pituitary adenoma, pituitary adenoma association due to defects in familial succinate dehydrogenase genes, and the recently identified X-linked acrogigantism. The early diagnosis of these conditions helps guide early intervention, screening, and genetic counseling of patients and their family members. In this review, we provide a concise and up-to-date discussion on the genetics of gigantism and acromegaly.
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PMID:Genetics of gigantism and acromegaly. 2765 86