• Asian Pacific Journal of Cancer Prevention
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• v.17 no.10
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• pp.4609-4614
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• 2016

Purpose: To evaluate the therapeutic effects of copper reduction on angiogenesis-related factors in patients with glioblastoma multiforme treated by gamma knife radiosurgery. Materials and Methods: In the present block randomized, placebo-controlled trial, fifty eligible patients with a diagnosis of glioblastoma multiforme who were candidates for gamma knife radiosurgery were randomly assigned into two groups to receive daily either 1gr penicillamine and a low copper diet or placebo for three months. The intervention started on the same day as gamma knife radiosurgery. Serum interleukin-6 (IL-6), tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$), vascular endothelial growth factor (VEGF) and copper levels were measured at baseline and after the intervention. The serum copper level was used as the final index of compliance with the diet. In order to control probable side effects of intervention, laboratory tests were conducted at the beginning, middle and end of the study. Results: The patients had a mean age and Karnofsky Performance Scale of 43.7 years and 75 respectively. Mean serum copper levels were significantly reduced in intervention group. Mean survival time was 18.5 months in intervention group vs. 14.9 in placebo group. VEGF and IL-6 levels in the intervention group were also significantly reduced compared to the placebo group and $TNF-{\alpha}$ increased less. Conclusions: It seems that reducing the level of copper in the diet and dosing with penicillamine leads to decline of angiogenesis-related factors such as VEGF, IL-6 and $TNF-{\alpha}$. Approaches targeting angiogenesis may improve survival and can be used as a future therapeutic strategy.

• Journal of Physiology & Pathology in Korean Medicine
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• v.16 no.5
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• pp.1066-1069
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• 2002

Wilson's disease is a rare inborn error of metabolism inherited as a autosomal recessive trait. The disease has varied mode of manifestations. It is characterized by different neurologic disorder and hepatic disease. I experienced a case of Wilson's disease in 40 year old woman who was suffered from liver cirrhosis, severe anorexia, and classical neurologic symptoms such as tremour, dysarthria and ataxia. The symptoms was not relieved by D-penicillamine, Youngyanggaksan and Samchulgunbi-tang but anorexia was improved significantly by same medication.

• Toxicological Research
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• v.11 no.1
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• pp.43-49
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• 1995

The effects of metallothionein and cadmium ion on the hepatic aldehyde oxidase activity and brain lipid peroxidation were tested in vitro. The content of brain lipid peroxide at the condition of normal or $300{\mu}M$ Fe(II)-induced was remarkably reduced by the addition of metallothionein in the incubation mixture. The induced content of lipid peroxide by cadmium $(30{\mu}g/ml)$ ion was reduced by metallothionein $(100{\mu}g/ml)$. The activity of aldehyde oxidase was not affected by metallothionein, but cadmium ion $(8.38{\mu}g/ml)$ increased the activity of aldehyde oxidase about 80% compared to the control. The cadmium-induced activity of aldehyde oxidase was restored to the control level by metallothionein or penicillamine.

• Archives of Pharmacal Research
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• v.25 no.5
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• pp.625-627
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• 2002

From the leaves of Brassica juncea, an radical scavenging isorhamnetin 7-Ο-glucoside on peroxynitrite and 1,1-diphenyl-2-picrylhydrazyl (DPPH) was isolated and characterized based on the spectroscopic evidence. The compound showed the peroxynitrite and DPPH scavenging activities with $IC_{50}$ values of 2.07$\pm$0.17 and 13.3 $\mu$M, respectively. Penicillamine and L-ascorbic acid as positive control exhibited radical scavenging activities with $IC_{50}$ values of 3.17$\pm$0.39 and 12.78 $\mu$M, respectively.

• Archives of Pharmacal Research
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• v.18 no.1
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• pp.56-59
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• 1995

The effect of ceruloplasmin or copper ion on hepatic xanthine oxidase activity and type conversion was investigated using rat liver in vitro. It was observed that ceruloplasmin increased xanthine oxidase type conversion depending on duration of its storage. Xanthine oxidase (type O) activity and type conversion in incubation mixture was increased by the addition of heated celuroplasmin in a temperature dependent manner. The type conversioin of xanthine oxidase induced by heated ceruloplasmin was retumed to normal by the tratment with DTT or penicillamine. The effect of copper ion on type conversion of xanthine oxidase was similar to that of heated ceruloplasmin.

• BMB Reports
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• v.28 no.6
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• pp.490-493
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• 1995

We investigated the effect of hydrogen peroxide-treated metallothionein on the hepatic xanthine oxidase activity in vitro. When the metallothionein was preincubated with 1 mM of hydrogen peroxide, the activity of xanthine oxidase and type conversion were elevated dose-dependently by the addition of metallothionein into the reaction mixture. While increasing the treatment of hydrogen peroxide to the $50{\mu}g$of metallothionein, the xanthine oxidase activity and type conversion ratio were remarkably elevated dose dependently compared to the control. When cadmium ion was added to the reaction mixture, the increasing pattern of the enzyme activity was similar to the effect of hydrogen peroxide-treated metallothionein. DTT or penicillamine restored the increasing activity and type conversion of xanthine oxidase by the cadmium ion to the control level.

• Journal of Pharmaceutical Investigation
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• v.21 no.4
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• pp.201-213
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• 1991

There are many drugs reproted to show unusual pharmacokinetic behavior by producing a significant secondary peak in the plasma concentration-time curve after oral administration. The drugs are ranitidine, cimetidine, acetaminophen, aspirin, furosemide, bumetanide, piretanide, veralipride, sobrerol, penicillamine and doxycycline etc. Enterohepatic circulation-, two absorption site-, biphasic gastric emptying-, tissue deposition- and multi-fraction absorption theories have been suggested for the mechanisms of this phenomenon. Here, the theories were reviewed and critisized for their validity as a possible mechanism of the multiple peak phenomenon.

• Childhood Kidney Diseases
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• v.27 no.2
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• pp.127-132
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• 2023

Cystinuria, a genetically inherited disorder, is a rare cause of kidney stones. It is characterized by impaired transport of cystine and amino acids in the proximal renal tubule and the small intestine. Most patients develop cystine stones throughout their lifetime. Recurrent renal stones need to be extracted by repeated urologic interventions. Treatment options of cystinuria for preventing stone recurrence are limited and poorly tolerated. In this study, we report a pediatric case of cystinuria with a heterozygous SLC3A1 mutation diagnosed by stone analysis, measurement of urine cystine excretion, and genetic analysis. There were recurrent renal stones despite repetitive shock wave lithotripsy and retrograde intrarenal surgery. However, the rate of stone formation seemed to be slower after D-penicillamine was added into adequate hydration and urinary alkalinization.

• Journal of Life Science
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• v.21 no.7
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• pp.961-968
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• 2011

The formation of reactive lipid aldehydes, 4-hydroxynonenal (HNE) is shown to be derived from fatty acid hydroperoxides through the oxidative process. Among its known effects in cytotoxicity, HNE has been implicated in apoptotic cell death. To delineate its putative role as a potential mediator, we investigated the mechanism by which HNE induces apoptosis of endothelial cells (ECs). The anti-proliferative effects of HNE were tested through MTT assay after exposure to various concentrations ($5\sim15\;{\mu}M$) of HNE. We observed apoptotic bodies with propidium iodide staining, and measured the HNE induction of endothelial apoptosis by flow cytometry assay. We observed that cells exposed to HNE for 24 hr resulted in increased poly(ADP-ribose) polymerase cleavage and up-regulation of Bax. Data on the HNE action strongly indicated the involvement of reactive species, namely, intracellular ROS, nitrite, and peroxynitrite. To obtain evidence on the implication of ROS and peroxynitrite in HNE-induced apoptosis, a ROS scavenger, N-acetylcysteine (NAC), and a peroxynitrite scavenger, penicillamine, were tested. Results clearly indicate that the induction of apoptosis by HNE was effectively inhibited by NAC and penicillamine. Based on the present data, we conclude that the endothelial apoptosis induced by HNE involves both ROS generation and peroxynitrite activity. Our new data could lead to a redefinition of HNE action on apoptosis in ECs.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.256-261
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• 2005

Scleroderma is a connective tissue disease of unknown etiology, but known as a kind of auto-immune disease. It is most common in women especially in $30{\sim}50$ years, and very rare in childhood. It can be classified into two main classes : localized scleroderma & systemic sclerosis. Localized type has better prognosis, and usually involves skin only, or in some cases, the muscle below, except inner organs. Systemic type involves skin, oral mucosa and major internal organs. Involving facial skin, we can see small and sharp nose, expressionless stare and narrow oral aperture. Usually they have Raynaud's phenomenon, and in progress, show mouth opening limitation and sclerosis of tongue and gingiva. It is called CREST syndrome showing calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangioectasia. Treatment of scleroderma is systemic and localized steroid therapy, use of collagen-link inhibitor (D-penicillamine), immune depressor and etc. Mouth opening limitation can be improved by mouth stretching exercise. We report a 6 years old boy, diagnosed with localized scleroderma who had mouth opening limitation. We could get additional mouth opening, and have done successful restorative treatment of mandibular and maxillary 1st and 2nd deciduous molar under deep sedation with nitrous oxide and enflurane.