Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:1.6.99.3 (diaphorase)
5,903 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report two brothers with a previously undescribed type of mitochondrial encephalomyopathy and associated aminoacidopathy. Both have growth failure, progressive intellectual decline, deafness, neurologic dysfunction, exercise intolerance, lactic acidosis, and abnormal plasma and cerebrospinal fluid amino acid levels (elevated levels of alanine and low levels of threonine, methionine, citrulline, tryptophan, ornithine, arginine, and lysine). A muscle biopsy specimen taken from the younger, more severely affected brother showed abnormal mitochondrial morphology. Activities of the following enzymes in cultured fibroblasts from both boys were normal: pyruvate dehydrogenase, pyruvate carboxylase, phosphoenolpyruvate carboxykinase, cytochrome oxidase, reduced nicotinamide-adenine dinucleotide-cytochrome c reductase, and succinate cytochrome c reductase. Fibroblast mitochondria from the younger boy showed undetectable (less than 1% of control values) adenosine triphosphate synthesis with pyruvate and malate, whereas adenosine triphosphate synthesis with succinate was 70% of control values. These data indicate probably deficient activity of complex I of the electron transport chain. The boys' mother has progressive neurosensory hearing loss; their sister is clinically normal. Both mother and sister have many of the biochemical abnormalities found in the boys. It is possible, but not proved, that this disorder is inherited through maternal mitochondria.
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PMID:Mitochondrial encephalomyopathy with associated aminoacidopathy in a male sibship. 273 99

We have examined the morphology and distribution of nicotinamide adenine dinucleotide phosphate diaphorase (NADPH-diaphorase) cells in the retina of the guinea pig. Two morphologically distinct classes of labelled cells were detected, one with larger, darkly labelled somata commonly located in the inner nuclear layer (INL: NDa cells) and the other with smaller, lightly labelled somata in the ganglion cell layer (GCL: NDb cells). The somata of NDb cells did not vary in diameter with eccentricity, whereas those of the NDa cells were smallest in the visual streak. The number of NDa cells was approximately 3,500, with a mean density of 26/mm2 and NDb cells numbered approximately 4,400, with a mean density of 33 mm2. NDa cells were distributed relatively uniformly across the retina, whereas NDb cells concentrated in the visual streak and were restricted to the superior half of the retina. In these features of morphology and distribution. NADPH-diaphorase neurones of the guinea pig retina are distinct from those observed in other species. It remains to be elucidated whether the diversity in the morphology and distribution of NADPH-diaphorase neurones between species reflects a diversity in their function.
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PMID:Distinct patterns of distribution among NADPH-diaphorase neurones of the guinea pig retina. 277 50

The pectoralis (pars thoracicus) of the domestic pigeon (Columba livia) is divisible into two anatomical parts, the pars sternobrachialis (SB) and the pars thoracobrachialis (TB). Innervation to this complex is from rostral and caudal branches of the brachial ventral cord. In four anesthetized pigeons, the distribution of muscle units associated with each nerve branch was mapped after prolonged stimulation of each nerve and subsequent analysis for muscle fiber glycogen. An additional three animals were used to analyze the morphology, distribution, and histochemical profiles of the muscle fibers in the SB and TB subregions. Fibers were characterized on the basis of their reactions for myofibrillar adenosine triphosphates (alkaline and acid preincubation) and reduced nicotinamide adenine dinucleotide diaphorase (NADH-D). The SB is primarily innervated by the rostral nerve branch and the TB by the caudal nerve branch. For two-thirds of the muscle's length, the SB is separated from the TB by an aponeurosis, the membrana intermuscularis (MI). SB and TB fibers located posteroventral to the caudal margin of the MI are innervated variously by both nerves. Two populations of fibers were recognized, distinguishable primarily by 1) fiber diameter and 2) density of the NADH-D reaction product. Compared to the TB, the SB possesses a higher average percentage of large fibers. Within the SB but not the TB the percentage of large fibers increases from deep to superficial. These data support our previous findings that the pars thoracicus of the pigeon is partitioned into at least two functional subunits, each with a potential for independent action on the wing during flight.
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PMID:Neuromuscular organization of the pectoralis (pars thoracicus) of the pigeon (Columba livia): implications for motor control. 278 25

Serotoninergic and cholinergic neurons are known to appear earlier in the ontogeny (day E12) of the murine gut than those containing substance P or vasoactive intestinal peptide (day E14). It has also been demonstrated that proliferating neural precursors coexist with mature neurons in developing enteric ganglia. These observations have led to the hypotheses that peptidergic neurons develop later than those that utilize small molecule neurotransmitters and that the activity of early developing neurons may affect the phenotypic expression of coexisting neuroblasts. As a partial test of these hypotheses we studied the phenotypic expression of neurons recognized by antisera to neuropeptide Y (NPY) and calcitonin gene-related peptide (CGRP), and of those visualized by the histochemical demonstration of reduced nicotinamide adenine dinucleotide phosphate (NADPH) diaphorase activity. NADPH diaphorase activity, which is coexpressed with NPY immunoreactivity in all submucosal and many myenteric neurons, was first found on day E11 in clusters of cells in the dorsal mesogastrium. These cells also expressed neurofilament reactivity and thus were developing along a neuronal lineage. Enteric neurons that expressed NADPH diaphorase activity were visualized in the stomach one day later, on day E12. At this time, NADPH diaphorase-containing cells could no longer be demonstrated in the dorsal mesogastrium. NPY immunoreactivity first appeared in the wall of the bowel on day E12, when it was seen in cells in the presumptive stomach. By day E13, the entire length of the bowel contained NPY-immunoreactive neurons. Cells that displayed NADPH diaphorase activity were found at this time at both ends of the alimentary tract, but did not appear in the ileum until day E18. In contrast, CGRP immunoreactivity could not be detected anywhere in the gut until day E17, but by day E18 all regions of the bowel contained CGRP-immunoreactive neurons. Endogenous 5-HT was first detected at day E16 in mucosal epithelial cells in all segments of the gut except the stomach, where it appeared at day E18. The NPY/NADPH diaphorase set of neurons thus develop before the acquisition of a detectable level of endogenous 5-HT or enteric neural 5-HT receptors (which arise in the foregut at day E14). These observations demonstrate that enteric neurons that express small molecule neurotransmitters do not necessarily develop earlier than peptidergic neurons as a class; however, various types of enteric neurons do appear in a sequential order.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Time course of expression of neuropeptide Y, calcitonin gene-related peptide, and NADPH diaphorase activity in neurons of the developing murine bowel and the appearance of 5-hydroxytryptamine in mucosal enterochromaffin cells. 278 79

This study has examined the development of cells in the rat retina which contain nicotinamide adenine dinucleotide phosphate (NADPH) diaphorase. NADPH-diaphorase cells were first detected at postnatal day (P) 3, in somata located in the inner part of the cytoblast layer (CBL). At this age, NADPH-diaphorase reactivity was also seen in weakly labelled fibers in the presumptive outer plexiform layer (OPL). By P5, the somata of most labelled cells were in the inner part of the inner nuclear layer (INL), and by P11, their processes had spread extensively within the inner plexiform layer (IPL). By P25, there was a striking change in the pattern of NADPH-diaphorase reactivity. First, cells had lost reactivity from their large and extensive dendrites and second, there was a distinct reduction in the diameters of labelled somata. Thus, NADPH-diaphorase reactivity was most prominent during the period of synaptogenesis in the IPL. Labelled cells at P3 numbered 120 and were largely found at the superior margin of the retina. By P11, their total number had increased to the adult value of about 3400 and their density was highest in peripheral retina. With further development, the differential expansion of the retina appeared to lower the peripheral densities, resulting in an approximately uniform distribution by adulthood.
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PMID:Development of NADPH-diaphorase cells in the rat's retina. 281 96

In vivo effects of tetrahydrocannabinols (THCs) and their eight monooxygenated metabolites on the hepatic microsomal drug-metabolizing enzymes in mice were studied. delta 8-THC and its metabolites (7 alpha-hydroxy-, 7 beta-hydroxy- and 7-oxo-delta 8-THC, and 8 alpha, 9 alpha- and 8 beta, 9 beta-epoxyhexahydrocannabinol) tended to increase the enzyme contents or activities except for 7 beta-hydroxy-delta 8-THC which affected the microsomal enzymes in a different manner between the single and subchronic treatments. Single administration (5 mg/kg, i.v.) of 7-oxo-delta 8-THC, 8 alpha, 9 alpha- and 8 beta, 9 beta-epoxyhexahydrocannabinol led to the significant increase in hepatic microsomal p-nitroanisole O-demethylase and aniline hydroxylase activities accompanying a significant increase in cytochrome P-450 content in hepatic microsomes. The same results were obtained with subchronic treatment of mice with these metabolites (5 mg/kg/d, i.v. for 7 d), although the effect of 8 beta, 9 beta-epoxyhexahydrocannabinol on cytochrome P-450 was not statistically significant. 7 beta-Hydroxy-delta 8-THC significantly increased nicotinamide adenine dinucleotide phosphate (NADPH)-cytochrome c reductase and aniline hydroxylase activities by single administration, while the metabolite significantly decreased the contents of cytochrome b5 and P-450 and p-nitrophenol uridine diphosphate-glucuronyltransferase activity by the subchronic treatment. In contrast, delta 9-THC and its metabolites (8 alpha-hydroxy-, 8 beta-hydroxy- and 8-oxo-delta 9-THC) did not significantly affect the microsomal enzymes by both treatments except that the single administration of 8 alpha-hydroxy-delta 9-THC and the subchronic treatment of delta 9-THC significantly decreased NADPH-cytochrome c reductase activity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:In vivo effects of tetrahydrocannabinols and their eight monooxygenated metabolites of the hepatic microsomal drug-metabolizing enzyme systems of mice. 283 34

A lattice of high oxidative metabolic activity occurs in the intermediate gray layer of the human, monkey, and cat superior colliculus. It is composed of a matrix of high enzyme activity that surrounds pale islands or bands of lower activity. In the human the pale bands are 300-400 micron wide while in the smaller colliculi of the monkey and cat they are 100-200 micron wide. The lattice was demonstrated by studying either cytochrome oxidase or succinate dehydrogenase. In the cat and monkey the lattice occurs at the same depth as the lattice of intense acetylcholinesterase activity, but the two lattices are not in spatial register. In the human the lattice of high oxidative metabolic activity is in the middle of the intermediate gray layer, whereas the lattice of intensely stained cholinesterase activity is at the base of this layer, but again the two lattices are not in spatial register. However, in the middle of the intermediate gray layer of the human, there are elongated islands and bands of very low acetylcholinesterase activity that coincide with the pale islands and bands of low cytochrome oxidase activity. An additional lattice of high enzyme activity occurs based on the enzyme nicotinamide dinucleotide phosphate (reduced form)-diaphorase. This lattice is prominent in the cat, occurs more faintly in the monkey, but did not appear to be present in the human. In the intermediate gray layer it had a high degree of overlap with the acetylcholinesterase lattice. The lattice of high oxidative metabolism contains loosely knit clusters of large multipolar cells containing high cytochrome oxidase activity and these cells do not occur in the pale islands. By contrast the cell bodies in the intermediate gray layer that contain either acetylcholinesterase or the diaphorase occur both between and within the patches of corresponding, high enzyme activity. It is suggested that the acetylcholinesterase and diaphorase lattices are mainly associated with afferent fibers while the lattice of high oxidative metabolism is mainly associated with intrinsic cells. The lattices occur in all mammals studied to date and appear to represent a fundamental principle in the organization of the mammalian colliculus. It is concluded that the lattices will provide a useful basis for further studies of the relationship between the many afferent and efferent modules thought to exist in this structure.
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PMID:Lattices of high histochemical activity occur in the human, monkey, and cat superior colliculus. 284 Jun 1

The effects of idebenone (CV-2619) and its metabolites on respiratory activity and lipid peroxidation in isolated brain mitochondria from rats and dogs were studied. CV-2619 was easily reduced by canine brain mitochondria in the presence of respiratory substrates. Reduced CV-2619 (2H-CV-2619) was rapidly oxidized through the cytochrome b chain, indicating that the compound functioned simply as an electron carrier of mitochondrial respiratory system. Both nicotinamide adenine dinucleotide (NADH)- and nicotinamide adenine dinucleotide phosphate (NADPH)-dependent lipid peroxidations were examined in canine brain mitochondria in the presence of adenosine diphosphate (ADP) and Fe3+. NADH-cytochrome c reductase activity was sensitive to NADPH-dependent lipid peroxidation. CV-2619 (10(-5)M) strongly inhibited both types of the lipid peroxidation reactions and protected the resultant inactivation of the NADH-cytochrome c reductase activity. Activities of succinate oxidase in rat and canine brain mitochondria were virtually unaffected by CV-2619 and its metabolites (10(-5)-10(-6) M). On the other hand, CV-2619 markedly suppressed the state 3 respiration in glutamate oxidation in a dose dependent manner without any effect on the state 4 respiration and the ADP/O ratio in intact rat brain mitochondria. The inhibitory effect of CV-2619 was also observed in NADH-cytochrome c reductase, but not in NADH-2,6-dichlorophenolindophenol (DCIP) and NADH-ubiquinone reductases in canine brain mitochondria. These facts and results of inhibitor analysis suggest that the action site of CV-2619 is NADH-linked complex I in the mitochondrial respiratory chain and is different from that of inhibitors of oxidative phosphorylation such as rotenone, oligomycin and 2,4-dinitrophenol. Finally, the above findings suggest that CV-2619 acts as an electron carrier in respiratory chains and functions as an antioxidant against membrane damage caused by lipid peroxidation in brain mitochondria. It appears likely that the inhibition of oxygen consumption caused by CV-2619 is related to the effect on non-respiratory systems such as lipid peroxidation which also consumes oxygen.
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PMID:Effects of idebenone (CV-2619) and its metabolites on respiratory activity and lipid peroxidation in brain mitochondria from rats and dogs. 287 Nov 47

Exposure of cultures of cortical cells from mouse to either of the endogenous excitatory neurotoxins quinolinate or glutamate resulted in widespread neuronal destruction; but only in the cultures exposed to quinolinate, an N-methyl-D-aspartate agonist, was there a striking preservation of the subpopulation of neurons containing the enzyme nicotinamide adenine dinucleotide phosphate diaphorase (NADPH-d). Further investigation revealed that neurons containing NADPH-d were also resistant to the toxicity of N-methyl-D-aspartate itself but were selectively vulnerable to the toxicity of either kainate or quisqualate. Thus, neurons containing NADPH-d may have an unusual distribution of receptors for excitatory amino acids, with a relative lack of N-methyl-D-aspartate receptors and a relative preponderance of kainate or quisqualate receptors. Since selective sparing of neurons containing NADPH-d is a hallmark of Huntington's disease, the results support the hypothesis that the disease may be caused by excess exposure to quinolinate or some other endogenous N-methyl-D-aspartate agonist.
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PMID:Neurons containing NADPH-diaphorase are selectively resistant to quinolinate toxicity. 287 22

Neuropeptide Y and somatostatin immunoreactive neurons and processes were examined in human striatum using both immunofluorescence and avidin biotin immunoperoxidase methods. Reduced nicotinamide adenine dinucleotide phosphate diaphorase activity was histochemically determined by the reduction of nitro blue tetrazolium. Immunofluorescence using a monoclonal anti-somatostatin antibody and a polyclonal anti-neuropeptide Y antibody, followed by diaphorase histochemistry, showed that these three neurochemical markers are co-localized in a single population of medium-sized aspiny intrinsic neurons. Cells were evenly distributed in clusters throughout the striatum, but fiber density was higher in the nucleus accumbens and ventromedial regions of the caudate and putamen. Double-stained reduced nicotinamide adenine dinucleotide phosphate diaphorase-acetylcholinesterase sections demonstrated that these neurons are located in zones of high acetylcholinesterase activity, often at the interface of these zones with regions of low enzyme activity. These biochemically distinctive neurons are uniquely situated to modulate activity between striatal compartments. Our findings provide new information about the modular organization of the striatum and extend these observations in human brain.
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PMID:Neuropeptide Y, somatostatin, and reduced nicotinamide adenine dinucleotide phosphate diaphorase in the human striatum: a combined immunocytochemical and enzyme histochemical study. 288 80


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