Evidence that the ubiquitin proteolytic pathway is involved in the degradation of precipitated globin chains in thalassaemia
Ultrastructural immunocytochemical studies were performed on sections of bone marrow from three patients with β‐thalassaemia major and two patients with haemoglobin H (HbH) disease. Some sections were reacted with either a polyclonal or a monoclonal anti‐human‐ubiquitin antibody and the reaction vis...
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| Veröffentlicht in: | British journal of haematology 1998-05, Vol.101 (2), p.245-250 |
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| creator | WICKRAMASINGHE, S. N LEE, M. J |
| description | Ultrastructural immunocytochemical studies were performed on sections of bone marrow from three patients with β‐thalassaemia major and two patients with haemoglobin H (HbH) disease. Some sections were reacted with either a polyclonal or a monoclonal anti‐human‐ubiquitin antibody and the reaction visualized using a gold‐labelled secondary antibody. The inclusions of precipitated globin chains found within the erythropoietic cells of all five patients reacted much more strongly than the surrounding inclusion‐free cytoplasm with both of the anti‐ubiquitin antibodies, indicating that the precipitated globin chains were ubiquitinated. A non‐specific reaction between the anti‐ubiquitin antibodies and the inclusions was excluded by demonstrating that various other antibodies, including a polyclonal anti‐human cathepsin D antibody, did not react with the inclusions. The data suggest that the ubiquitin proteolytic pathway is involved in the degradation of precipitated globin chains in α‐ and β‐thalassaemia. |
| doi_str_mv | 10.1046/j.1365-2141.1998.00699.x |
| format | Article |
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N ; LEE, M. J</creator><creatorcontrib>WICKRAMASINGHE, S. N ; LEE, M. J</creatorcontrib><description>Ultrastructural immunocytochemical studies were performed on sections of bone marrow from three patients with β‐thalassaemia major and two patients with haemoglobin H (HbH) disease. Some sections were reacted with either a polyclonal or a monoclonal anti‐human‐ubiquitin antibody and the reaction visualized using a gold‐labelled secondary antibody. The inclusions of precipitated globin chains found within the erythropoietic cells of all five patients reacted much more strongly than the surrounding inclusion‐free cytoplasm with both of the anti‐ubiquitin antibodies, indicating that the precipitated globin chains were ubiquitinated. A non‐specific reaction between the anti‐ubiquitin antibodies and the inclusions was excluded by demonstrating that various other antibodies, including a polyclonal anti‐human cathepsin D antibody, did not react with the inclusions. The data suggest that the ubiquitin proteolytic pathway is involved in the degradation of precipitated globin chains in α‐ and β‐thalassaemia.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1046/j.1365-2141.1998.00699.x</identifier><identifier>PMID: 9609517</identifier><identifier>CODEN: BJHEAL</identifier><language>eng</language><publisher>Oxford, U.K. and Cambridge, USA: Blackwell Science Ltd</publisher><subject>Anemias. Hemoglobinopathies ; beta-Thalassemia - metabolism ; Biological and medical sciences ; cathepsin D ; Cathepsin D - metabolism ; Diseases of red blood cells ; Globins - metabolism ; Hematologic and hematopoietic diseases ; Hematology ; Humans ; Immunohistochemistry ; Medical sciences ; Microscopy, Electron ; precipitated globin chains ; proteolysis ; thalassaemia ; ubiquitin ; Ubiquitins - metabolism</subject><ispartof>British journal of haematology, 1998-05, Vol.101 (2), p.245-250</ispartof><rights>1998 INIST-CNRS</rights><rights>Copyright Blackwell Scientific Publications Ltd. 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N</creatorcontrib><creatorcontrib>LEE, M. J</creatorcontrib><title>Evidence that the ubiquitin proteolytic pathway is involved in the degradation of precipitated globin chains in thalassaemia</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>Ultrastructural immunocytochemical studies were performed on sections of bone marrow from three patients with β‐thalassaemia major and two patients with haemoglobin H (HbH) disease. Some sections were reacted with either a polyclonal or a monoclonal anti‐human‐ubiquitin antibody and the reaction visualized using a gold‐labelled secondary antibody. The inclusions of precipitated globin chains found within the erythropoietic cells of all five patients reacted much more strongly than the surrounding inclusion‐free cytoplasm with both of the anti‐ubiquitin antibodies, indicating that the precipitated globin chains were ubiquitinated. A non‐specific reaction between the anti‐ubiquitin antibodies and the inclusions was excluded by demonstrating that various other antibodies, including a polyclonal anti‐human cathepsin D antibody, did not react with the inclusions. The data suggest that the ubiquitin proteolytic pathway is involved in the degradation of precipitated globin chains in α‐ and β‐thalassaemia.</description><subject>Anemias. Hemoglobinopathies</subject><subject>beta-Thalassemia - metabolism</subject><subject>Biological and medical sciences</subject><subject>cathepsin D</subject><subject>Cathepsin D - metabolism</subject><subject>Diseases of red blood cells</subject><subject>Globins - metabolism</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Hematology</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Medical sciences</subject><subject>Microscopy, Electron</subject><subject>precipitated globin chains</subject><subject>proteolysis</subject><subject>thalassaemia</subject><subject>ubiquitin</subject><subject>Ubiquitins - metabolism</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1998</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkUGP0zAQhS0EWsrCT0CKEOKWME5S2yNxgdXCglbiAmdr4ky2rtKkGzvdrcSPx91WPXDiYo_0vvfs0RMik1BIqNXHdSErtcxLWctCIpoCQCEWj8_E4iw8FwsA0HkymJfiVQhrAFnBUl6IC1SAS6kX4s_1zrc8OM7iimI6OJsbfz_76IdsO42Rx34fvcu2FFcPtM98yPywG_sdt2l4MrR8N1FL0Y9DNnbJxc5vfaSYkLt-bBLmVuSHcDRQTyEQbzy9Fi866gO_Od2X4vfX619XN_ntz2_frz7f5q5Oy-XYkHbKaQOqNKbkGnW7NBo0SZYSdIsdEaplzW3TINaAdU0oDSuFWrWyuhQfjrlpofuZQ7QbHxz3PQ08zsFqREjBkMB3_4DrcZ6G9Dcr0SgwUJcJMkfITWMIE3d2O_kNTXsrwR7asWt7KMEeSrCHduxTO_YxWd-e8udmw-3ZeKoj6e9POgVHfTfR4Hw4Y2VZVVhVCft0xB58z_v_ft5--XGThuovJlyrWQ</recordid><startdate>199805</startdate><enddate>199805</enddate><creator>WICKRAMASINGHE, S. 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Hemoglobinopathies</topic><topic>beta-Thalassemia - metabolism</topic><topic>Biological and medical sciences</topic><topic>cathepsin D</topic><topic>Cathepsin D - metabolism</topic><topic>Diseases of red blood cells</topic><topic>Globins - metabolism</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Hematology</topic><topic>Humans</topic><topic>Immunohistochemistry</topic><topic>Medical sciences</topic><topic>Microscopy, Electron</topic><topic>precipitated globin chains</topic><topic>proteolysis</topic><topic>thalassaemia</topic><topic>ubiquitin</topic><topic>Ubiquitins - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>WICKRAMASINGHE, S. N</creatorcontrib><creatorcontrib>LEE, M. 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The inclusions of precipitated globin chains found within the erythropoietic cells of all five patients reacted much more strongly than the surrounding inclusion‐free cytoplasm with both of the anti‐ubiquitin antibodies, indicating that the precipitated globin chains were ubiquitinated. A non‐specific reaction between the anti‐ubiquitin antibodies and the inclusions was excluded by demonstrating that various other antibodies, including a polyclonal anti‐human cathepsin D antibody, did not react with the inclusions. The data suggest that the ubiquitin proteolytic pathway is involved in the degradation of precipitated globin chains in α‐ and β‐thalassaemia.</abstract><cop>Oxford, U.K. and Cambridge, USA</cop><pub>Blackwell Science Ltd</pub><pmid>9609517</pmid><doi>10.1046/j.1365-2141.1998.00699.x</doi><tpages>6</tpages></addata></record> |
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| ispartof | British journal of haematology, 1998-05, Vol.101 (2), p.245-250 |
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| source | MEDLINE; Wiley Online Library Journals Frontfile Complete; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Wiley Free Content |
| subjects | Anemias. Hemoglobinopathies beta-Thalassemia - metabolism Biological and medical sciences cathepsin D Cathepsin D - metabolism Diseases of red blood cells Globins - metabolism Hematologic and hematopoietic diseases Hematology Humans Immunohistochemistry Medical sciences Microscopy, Electron precipitated globin chains proteolysis thalassaemia ubiquitin Ubiquitins - metabolism |
| title | Evidence that the ubiquitin proteolytic pathway is involved in the degradation of precipitated globin chains in thalassaemia |
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