caa a22 4500 477118526 CHVBK 20180405111629.0 cr unu---uuuuu 170330e19960301xx s 000 0 eng 10.1007/BF00239315 doi (NATIONALLICENCE)springer-10.1007/BF00239315 Molecular basis of pathophysiology of Indian childhood cirrhosis: Role of nuclear copper accumulation in liver [Elektronische Daten] [Rajendra Prasad, Gurjeet Kaur, Ravindra Nath, B.N.S. Walia] Indian Childhood Cirrhosis (ICC) is a disease of abnormal copper metabolism commonly characterized by swelling and degeneration of liver cells along with the presence of orcein staining deposits of copper. Hepatic copper content of ICC patients was about 43 fold higher than those of control subjects. The data on sub-cellular distribution of copper revealed massive accumulation of Copper (73%) of total cell copper) in the nuclear fraction (455 μg Cu/g tissue nuclei). On further distribution of copper in nuclear fraction, the enrichment of copper in heterochromatin and euchromatin of ICC nuclei was found to be 48 and 15 fold higher over control fractions respectively. The ultra-violet spectra of heterochromatin and euchromatin isolated from ICC nuclear fraction showed a broad absorption maxima as compared to controls. Further, A260/A280 ratio was markedly lower in heterochromatin and euchromatin of ICC liver in comparison to controls. An antioxidant enzyme, catalase activity was also significantly reduced in ICC liver as compared to control. Further, DNA fragmentation studies indicated that there was significantly increased DNA fragmentation in ICC liver. Collectively, these findings suggest that massive accumulation of copper in nucleus and decrease in catalase activity was associated with DNA fragmentation in hepatocyte of ICC disease. Kluwer Academic Publishers, 1996 Indian childhood cirrhosis nationallicence hetero and euchromatin nationallicence copper nationallicence catalase nationallicence DNA fragmentation nationallicence ICC : Indian Childhood Cirrhosis nationallicence Prasad Rajendra Department of Biochemistry, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India aut Kaur Gurjeet Department of Biochemistry, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India aut Nath Ravindra Department of Biochemistry, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India aut Walia B.N.S. Department of Paediatrics, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India aut Molecular and Cellular Biochemistry Kluwer Academic Publishers 156/1(1996-03-01), 25-30 0300-8177 156:1<25 1996 156 11010 https://doi.org/10.1007/BF00239315 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00239315 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Prasad Rajendra Department of Biochemistry, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India aut NATIONALLICENCE 700 1- Kaur Gurjeet Department of Biochemistry, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India aut NATIONALLICENCE 700 1- Nath Ravindra Department of Biochemistry, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India aut NATIONALLICENCE 700 1- Walia B.N.S. Department of Paediatrics, Postgraduate Institute of Medical Education and Research, 160012, Chandigarh, India aut NATIONALLICENCE 773 0- Molecular and Cellular Biochemistry Kluwer Academic Publishers 156/1(1996-03-01), 25-30 0300-8177 156:1<25 1996 156 11010 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer