Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:1.16.3.1 (ceruloplasmin)
 5,074 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Some organophosphorous esters cause a polyneuropathy which becomes clinically evident 2 weeks after a single dose. The pathogenesis involves modifications of a target protein, neuropathy target esterase, in the axons and a selective inhibition of retrograde axonal transport. It was suggested that copper metabolism might also be involved because of increased levels of plasma copper and ceruloplasmin in animals developing this polyneuropathy. Our results do not confirm this observation; treatment of hens with highly neuropathic single doses of two organophosphates (dihexyl-2,2-dichlorovinyl phosphate and mono-o-cresyl diphenyl phosphate) does not affect total and plasma free copper when measured several times during the development of polyneuropathy. We concluded that copper homeostasis is not affected and that copper changes are unlikely to be involved in the pathogenesis of this polyneuropathy.
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PMID:Blood copper in organophosphate-induced delayed polyneuropathy. 283 31

Clinical findings of ocular involvement in two patients with familial amyloidotic polyneuropathy are described. Both cases revealed irregular pupillary margin, white membranous material on the pupillary border and on the lens surface. Open-angle glaucoma was found in one case, and ocular hypertension in the other. Histopathological examination of the tissues obtained during trabeculectomy in Case 1 revealed a large amount of amyloid substance around the vessels of the conjunctiva and the iris and in the endothelial meshwork of the chamber angle. Electron microscopic observation revealed that amyloid fibrils had formed in the basement membrane of the endothelial cells of the blood vessels in the conjunctiva and the iris. However, no amyloid fibrils were observed in the endothelial cells of the trabecular meshwork. Crossed immunoelectrophoresis of the aqueous humor of Case 1 showed a high-protein content, especially of alpha 1-lipoprotein and ceruloplasmin. The findings described above suggest that glaucoma or ocular hypertension in association with familial amyloidotic polyneuropathy is a result of the accumulation of amyloid substance in the endothelial meshwork.
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PMID:Familial amyloidotic polyneuropathy: ocular manifestations with clinicopathological observation. 609 57

Axon degeneration accompanying its demielinization is a main course of neurological insufficiency typical for GBS. The mechanisms of axon degeneration, considered as the secondary result of serve inflammation are not established. We aimed to determine the role of oxidative metabolism in viral polyneuropathy pathogenesis. The activity of pro- and antioxidant systems of the body was studied by electron paramagnetic resonance (EPR) method. In blood and cerebrospinal fluid the intensive EPR signals of nitric oxide (NO), complexes of NO with nonhemic iron (HbNO), lypo- and superoxide radicals content noticeably increases, the signals of free Mn2+ and Fe2+ revealed, the activity of blood antioxidant enzymes, ceruloplasmin and katalasa increases (by 60%), superoxidedismitase's and glutation reductases activity decreases (by 20% and 70% correspondingly). It was considered, that inflammatory damage of nervous system induced by different infectious stimulus is initiated by activated immune cell proinflamatory agents (reactive oxygen and nitrogen species). Subsequently the oxidative stress, as result of accumulation of generators of reactive oxygen species, disordered intracellular metabolism products, contributes to axon demielinization and degeneration.
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PMID:[The role of oxidative stress in pathogenesis of GBS]. 1717 87

Acquired copper deficiency constitutes an under-recognised cause of myelopathy. Aim of the study was to describe the clinical and imaging features at admission and after copper supplementation of a patient with acquired copper deficiency myeloneuropathy. A 73-year-old woman presented with anaemia and signs of posterior column dysfunction. Somatosensory evoked potentials showed impaired central pathway conduction. Serum copper and caeruloplasmin levels were low. Nerve conduction assessment revealed axonal polyneuropathy. Spinal magnetic resonance imaging (MRI) showed posterior column hyperintensity. Diffusion tensor imaging disclosed decreased fractional anisotropy (FA) corresponding to the hyperintensity. Copper supplementation normalised the haematological picture, whereas vibratory sensitivity was only slightly improved. Control MRI revealed a slight hyperintensity at C1-C2 level; FA values normalised. In conclusion, in acquired copper-deficiency-associated myelopathy, correction of blood and MRI alterations precedes that of neurological manifestations, which may remain suboptimal.
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PMID:Myeloneuropathy due to copper deficiency: clinical and MRI findings after copper supplementation. 1976 78