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:1.4.3.13 (lysyl oxidase)
1,248 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Copper therapy was applied to brindled mouse mutants, which suffer from lethal hypocupraemia, by using cuprous and cupric solutions. The method of treatment was a single subcutaneous injection of 50 microgram of copper at 7 days of age. Early effects of the dose were: prevention of the tremors and spasms seen in untreated mutants, raising to normal and near-normal of caeruloplasmin oxidase and lysyl oxidase activities and pigmentation of skin and fur. Growth of mutants was retarded up to 23 days of age, but thereafter they rapidly gained weight to be nearly normal by 60 days of age. At 3 days after injection, copper concentrations in previously deficient mutant organs apart from liver were at least as much as those of treated normals, which had remained unchanged. Copper in mutant livers had increased only slightly in comparison with the normal control. A state of copper deficiency recurred in mutant tissues by 25 days after injection. A solution of Cu+, retained as such by an alkyl polyether, and sebacic acid resulted in greater growth rates after 23 days than did three other copper treatments. Cu+ may have resulted in an improved growth response owing to it being more readily metabolized than C12+. Delayed release of copper from the site of injection may have played an important role.
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PMID:Copper metabolism in mottled mouse mutants: copper therapy of brindled (Mobr) mice. 57 18

Occipital horn syndrome (OHS; Ehlers-Danlos syndrome type IX) belongs to the category of the copper metabolism disorders and is at present being investigated biochemically as is Menkes disease. We report a case of OHS in a 34-year-old male, which we believe to be the first Japanese case. He had been noted to have psychomotor retardation since his early childhood and now presents severe psychomotor retardation and muscle atrophy. He shows characteristic facial appearance, hyperelasticity of the skin, joint subluxation and generalized muscular atrophy. Laboratory investigations revealed a low serum copper and ceruloplasmin level as well as intestinal non-absorption of copper. Radiologic imagings showed occipital exostoses and bladder diverticula. The activity of lysyl oxidase, a copper-dependent enzyme involved in cross-link formation in collagen, was decreased in a skin-biopsied specimen. Electronmicroscopic investigation of a muscle biopsy showed irregularity of the myofibrillar network and accumulation of the concentric laminated bodies in the subsarcolemmal regions.
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PMID:[Occipital horn syndrome (Ehlers-Danlos syndrome type IX) with severe psychomotor retardation and muscle atrophy--a first Japanese case]. 168 78

Effects of cadmium (Cd) on lysyl oxidase activity and copper (Cu) metabolism in bone were studied using Cu-deficient rats supplemented with Cu and/or Cd in a diet. When fed for 8 weeks on a diet containing 0.3 ppm or less Cu (-Cu diet), weanling rats revealed anemia, and markedly decreased plasma ceruloplasmin activity and serum Cu to less than 15% of normal level, showing features of Cu-deficiency. These rats were divided into four groups and refed for another 2 weeks on the following diets: Group I, -Cu diet; Group II, -Cu diet with 50 ppm Cd (+Cd diet); Group III, -Cu diet supplemented with 15 ppm Cu (+Cu diet); group IV, -Cu diet with both Cu and Cd (+Cu/+Cd diet). After 2 weeks, serum Cu levels of Groups I, II, III and IV were 1.8, 0.8, 78 and 74% of the normal control level (1.438 +/- 0.060 micrograms/ml), respectively. Concentrations of Cu in epi- and metaphyses of the control group, Groups I, II, III and IV were 1.45 +/- 0.20, 0.67 +/- 0.08, 0.76 +/- 0.12, 1.40 +/- 0.31 and 1.22 +/- 0.05 micrograms/g wet tissue, in that order. Concentrations of Cd in epi- and metaphysis increased in only Groups II and IV and were 0.15 +/- 0.03 and 0.18 +/- 0.01 micrograms/g wet tissue, respectively. Thus, having both Cd and Cu supplements in a diet did not inhibit each other's uptake into the tissue.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effect of dietary cadmium and/or copper on the bone lysyl oxidase in copper-deficient rats relative to the metabolism of copper in the bone. 197 55

The biochemistry of the essential trace element copper has been outlined. Following absorption, Cu(II) is transported by serum albumin and transcuprein to the liver where it is incorporated into the plasma Cu-protein, ceruloplasmin, or, possibly, stored as Cu-metallothionein or as superoxide dismutase. Ceruloplasmin is the long-term copper transporter and carries Cu(II) to the tissues for the biosynthesis of key Cu(II) enzymes, especially cytochrome c oxidase, lysyl oxidase and others. The production of copper enzymes raises many new questions about the metabolism of copper. Since ceruloplasmin is the centerpiece of copper metabolism and function, we conclude with more details on its chemistry and multifunctions. This Cu-protein of 132,000 daltons has now been totally sequenced and the copper-containing active sites located. Finally, we have proposed seven possible functions for ceruloplasmin, and there is now good evidence for the existence of ceruloplasmin receptors to expedite some of these functions.
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PMID:Perspectives on copper biochemistry. 300 68

Copper is required in trace amounts for many body functions. The prominent effects of Cu deficiency or Cu toxicosis differs greatly between animal species. Along with iron, Cu is necessary for the transfer of O2 via a cascade of enzymes so that energy may be available for vital body functions without overheating of the tissues through rapid oxidation. As a part of lysyl oxidase, Cu has an obligate function in the maturation of all connective tissue (including elastic tissue and bone) maintaining the form and integrity of all body organs. As a constituent of tyrosinase, Cu is involved in the formation of melanin, thus preventing albinism. Copper also is involved in the myelination of nerve fibers and the production of neutrophils, enkephalins, lipoproteins, and cholesterol. Copper must be properly sequestered to prevent toxicosis. Copper is stored primarily as metallothioneins and as superoxide dismutase and is transported primarily as ceruloplasmin or as low molecular weight proteins, peptides, and amino acids.
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PMID:Comparative metabolism of copper. 355 17

Copper's role in connective tissue is linked to the enzyme lysyl oxidase. From a biochemical perspective, copper is a cofactor for the enzyme and a determinant of its activity in connective tissues. Lysyl oxidase catalyses a post-translational oxidation of certain lysine and hydroxylysine residues. The peptidyl aldehydes so formed become active centres for the formation of cross-links in collagen and elastin. Less well understood is how copper controls the steady-state activity of lysyl oxidase; the enzyme fails in copper deficiency. Giving copper to a deprived animal increases lysyl oxidase activity in aortic tissue. Such activation in vivo appears to require caeruloplasmin. Suspending aortic tissue in a copper-enriched growth medium also activates lysyl oxidase provided that tissue structure is kept intact. Activation in vitro occurs with the binding of copper to a large-molecular-weight component, presumably the enzyme. Binding will not occur if protein synthesis is blocked. These studies clearly show that the synthesis of mature elastin and collagen can be controlled by the availability of copper. They further suggest that transport of copper to aortic tissue and its engagement to lysyl oxidase are linked to the synthesis or lysyl oxidase, an extracellular carrier, or both.
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PMID:Copper and the synthesis of elastin and collagen. 611 May 24

Copper deficiency can reduce the productivity of livestock. The effect of copper deficiency on a number of copper enzymes and copper-dependent systems is discussed, to highlight the areas where their role needs to be clarified. Special reference is made to cytochrome c oxidase, lysyl oxidase, superoxide dismutase and endoplasmic reticulum enzymes and to their role in the expression of disease. The modification of microbiological insult by a change in superoxide dismutase activity without any other direct metabolic consequences is discussed, to introduce the concept of an external challenge being necessary before any effect of an otherwise sub-clinical copper deficiency is observed. The changes in activity of the various copper enzymes are described in clinical and experimentally induced copper deficiency in sheep and cattle, two species in which copper deficiency can have economic consequences. The diagnostic value of various blood markers, such as copper, caeruloplasmin and erythrocyte superoxide dismutase is discussed. The measured degree of hypocupraemia is related to different types of sampling (e.g. plasma or serum), physiological status (e.g. in the pre- and postpartum cow), changes that occur in the neonate, and also to the effect of the acute-phase reaction. The use of erythrocyte superoxide dismutase as a marker for the copper status of sheep and cattle is compared with more conventional markers such as plasma concentration of copper. The use of blood markers to map the extent and location of hypocupraemia (due to reduced copper intake or availability) among suckler (beef) herds in Northern Ireland is also discussed.
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PMID:Copper deficiency in ruminants. 611 May 25

We have studied the effect of L-ascorbic acid (vitamin C) on copper metabolism using copper-deficient chicks and the activation of lysyl oxidase copper-dependent enzyme, to assess bioavailability of copper. When administered intraperitoneally with (or 75 minutes before) CuSO4, L-ascorbate significantly impared the effectiveness of copper to restore lysyl oxidase activity in deficient chicks. L-ascorbate given 75 minutes after CuSO4 (i.e., in the post-absorption period), however, produced a substantial increase in copper-induced enzyme activation. L-ascorbate by itself showed no direct stimulating effect in deficient chicks. When the L-ascorbate was given to chicks that had received adequate dietary copper, there was a strong rise in ceruloplasmin and a slight, but significant increase in lysyl oxidase. An increase in ceruloplasmin in response to copper was also seen in deficient chicks and L-ascorbate also augmented that increase. Substituting D-isoascorbic acid for antagonistic properties of L-ascorbic acid on copper metabolism, but they also reveal possible sterospecific postabsorption roles for L-ascorbate in the metabolism of copper.
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PMID:A postabsorption effect of L-ascorbic acid on copper metabolism in chicks. 611 9

Because high levels of dietary zinc are known to reduce copper body stores, the objective was to determine if a high zinc maternal diet could induce a copper deficiency in the newborn pig fed a dried skim milk--glucose--starch diet unsupplemented with copper. The offspring of gilts, which were fed 5000 ppm of zinc, were allowed to nurse until 3 to 5 days of age when they were weaned and placed in individual stainless-steel pens. The dietary treatments were 0, 5 and 10 ppm added copper from copper sulfate. After 14 days, pigs receiving the 0-ppm copper diet weighed significantly less (P less than 0.05) and had reduced hemoglobin, hematocrit and serum copper concentrations and no detectable ceruloplasmin activity. After 5 weeks, the pigs were killed, and tissues were collected. The unsupplemented group had 16.4% of the aortic lysyl oxidase activity of the 5-ppm group. Cytochrome c oxidase activity in the heart and liver, and copper stores in the heart, liver, pancreas and kidney were depressed (P less than 0.05) in unsupplemented pigs compared to those receiving 5 ppm copper. These data demonstrate that it is possible to produce quickly a markedly copper-deficient pig, by using the offspring of sows fed 5000 ppm zinc, and support previous conclusions that the dietary copper requirement of the baby pig is about 5 ppm.
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PMID:A copper deficiency in neonatal pigs induced by a high zinc maternal diet. 613 53

Of 8 Thoroughbred foals in which osteochondrosis developed before weaning, 7 had serum copper and ceruloplasmin concentrations below normal. Three foals on one farm had serum zinc content high enough to suggest zinc toxicosis, and the liver of each foal contained abnormally high content of zinc. Four foals from the second farm had extremely low serum copper content, but normal serum zinc content. Evidence of environmental exposure to excess zinc was not found on either farm. The lesions in the zones of endochondral ossification of the afflicted foals were similar in many respects to those found in other species of animals with molybdenum-induced copper deficiency and with inhibition of the function of copper-dependent lysyl oxidase by beta-aminopropionitrile, a toxic component of Lathyrus odoratus known to cause osteolathyrism.
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PMID:Considerations of copper metabolism in osteochondrosis of suckling foals. 674 86


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