Deficient mitophagy pathways in sickle cell disease
Summary Sickle cell disease (SCD) is characterised by chronic haemolysis and oxidative stress. Herein, we investigated 30 SCD patients and found 40% with elevated mitochondria levels (SS‐mito+) in their mature red blood cells, while 60% exhibit similar mitochondria levels compared to the AA group (S...
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| Veröffentlicht in: | British journal of haematology 2021-06, Vol.193 (5), p.988-993 |
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| container_title | British journal of haematology |
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| creator | Martino, Suella Arlet, Jean‐Benoit Odièvre, Marie‐Hélène Jullien, Vincent Moras, Martina Hattab, Claude Lefebvre, Thibaud Gouya, Laurent Ostuni, Mariano A. Lefevre, Sophie D. Le Van Kim, Caroline |
| description | Summary
Sickle cell disease (SCD) is characterised by chronic haemolysis and oxidative stress. Herein, we investigated 30 SCD patients and found 40% with elevated mitochondria levels (SS‐mito+) in their mature red blood cells, while 60% exhibit similar mitochondria levels compared to the AA group (SS‐mito−). The SS‐mito+ patients are characterised by higher reticulocytosis and total bilirubin levels, lower foetal haemoglobin, and non‐functional mitochondria. Interestingly, we demonstrated decreased levels of mitophagy inducers, PINK1 and NIX, and higher levels of HSP90 chaperone in their red cells. Our results highlighted for the first time an abnormal retention of mitochondria in SCD linked with mitophagy‐related proteins. |
| doi_str_mv | 10.1111/bjh.17416 |
| format | Article |
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Sickle cell disease (SCD) is characterised by chronic haemolysis and oxidative stress. Herein, we investigated 30 SCD patients and found 40% with elevated mitochondria levels (SS‐mito+) in their mature red blood cells, while 60% exhibit similar mitochondria levels compared to the AA group (SS‐mito−). The SS‐mito+ patients are characterised by higher reticulocytosis and total bilirubin levels, lower foetal haemoglobin, and non‐functional mitochondria. Interestingly, we demonstrated decreased levels of mitophagy inducers, PINK1 and NIX, and higher levels of HSP90 chaperone in their red cells. Our results highlighted for the first time an abnormal retention of mitochondria in SCD linked with mitophagy‐related proteins.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1111/bjh.17416</identifier><identifier>PMID: 33754349</identifier><language>eng</language><publisher>England: Blackwell Publishing Ltd</publisher><subject>Bilirubin ; Erythrocytes ; Hematology ; Hemoglobin ; Hsp90 protein ; Mitochondria ; Mitophagy ; Oxidative stress ; PINK1/NIX ; PTEN-induced putative kinase ; Sickle cell anemia ; Sickle cell disease</subject><ispartof>British journal of haematology, 2021-06, Vol.193 (5), p.988-993</ispartof><rights>2021 British Society for Haematology and John Wiley & Sons Ltd</rights><rights>2021 British Society for Haematology and John Wiley & Sons Ltd.</rights><rights>Copyright © 2021 John Wiley & Sons Ltd</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4546-a761cd3fff93d0687fb32540756482979deb5da54e658b6c6a76ab4d15745a503</citedby><cites>FETCH-LOGICAL-c4546-a761cd3fff93d0687fb32540756482979deb5da54e658b6c6a76ab4d15745a503</cites><orcidid>0000-0002-7504-1881 ; 0000-0001-6769-3786 ; 0000-0002-3251-1310</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fbjh.17416$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fbjh.17416$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1416,1432,27923,27924,45573,45574,46408,46832</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/33754349$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Martino, Suella</creatorcontrib><creatorcontrib>Arlet, Jean‐Benoit</creatorcontrib><creatorcontrib>Odièvre, Marie‐Hélène</creatorcontrib><creatorcontrib>Jullien, Vincent</creatorcontrib><creatorcontrib>Moras, Martina</creatorcontrib><creatorcontrib>Hattab, Claude</creatorcontrib><creatorcontrib>Lefebvre, Thibaud</creatorcontrib><creatorcontrib>Gouya, Laurent</creatorcontrib><creatorcontrib>Ostuni, Mariano A.</creatorcontrib><creatorcontrib>Lefevre, Sophie D.</creatorcontrib><creatorcontrib>Le Van Kim, Caroline</creatorcontrib><title>Deficient mitophagy pathways in sickle cell disease</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>Summary
Sickle cell disease (SCD) is characterised by chronic haemolysis and oxidative stress. Herein, we investigated 30 SCD patients and found 40% with elevated mitochondria levels (SS‐mito+) in their mature red blood cells, while 60% exhibit similar mitochondria levels compared to the AA group (SS‐mito−). The SS‐mito+ patients are characterised by higher reticulocytosis and total bilirubin levels, lower foetal haemoglobin, and non‐functional mitochondria. Interestingly, we demonstrated decreased levels of mitophagy inducers, PINK1 and NIX, and higher levels of HSP90 chaperone in their red cells. Our results highlighted for the first time an abnormal retention of mitochondria in SCD linked with mitophagy‐related proteins.</description><subject>Bilirubin</subject><subject>Erythrocytes</subject><subject>Hematology</subject><subject>Hemoglobin</subject><subject>Hsp90 protein</subject><subject>Mitochondria</subject><subject>Mitophagy</subject><subject>Oxidative stress</subject><subject>PINK1/NIX</subject><subject>PTEN-induced putative kinase</subject><subject>Sickle cell anemia</subject><subject>Sickle cell disease</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><recordid>eNp10D1PwzAQBmALgWgpDPwBFIkFhrR27LOTEcpHQZVYYLacxCEu-SJOVOXf45LCgMQttzz36vQidE7wnLhZxJt8TgQj_ABNCeXgB4SRQzTFGAufYBZO0Im1G4wJxUCO0YRSAYyyaIronc5MYnTVeaXp6iZX74PXqC7fqsF6pvKsST4K7SW6KLzUWK2sPkVHmSqsPtvvGXp7uH9drvz1y-PT8mbtJwwY95XgJElplmURTTEPRRbTABgWwFkYRCJKdQypAqY5hDFPuDtQMUsJCAYKMJ2hqzG3aevPXttOlsbuHlGVrnsrA8CMQhCBcPTyD93UfVu575yiNAhCTkKnrkeVtLW1rc5k05pStYMkWO6alK5J-d2ksxf7xD4udforf6pzYDGCrSn08H-SvH1ejZFfvNB6TA</recordid><startdate>202106</startdate><enddate>202106</enddate><creator>Martino, Suella</creator><creator>Arlet, Jean‐Benoit</creator><creator>Odièvre, Marie‐Hélène</creator><creator>Jullien, Vincent</creator><creator>Moras, Martina</creator><creator>Hattab, Claude</creator><creator>Lefebvre, Thibaud</creator><creator>Gouya, Laurent</creator><creator>Ostuni, Mariano A.</creator><creator>Lefevre, Sophie D.</creator><creator>Le Van Kim, Caroline</creator><general>Blackwell Publishing Ltd</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-7504-1881</orcidid><orcidid>https://orcid.org/0000-0001-6769-3786</orcidid><orcidid>https://orcid.org/0000-0002-3251-1310</orcidid></search><sort><creationdate>202106</creationdate><title>Deficient mitophagy pathways in sickle cell disease</title><author>Martino, Suella ; Arlet, Jean‐Benoit ; Odièvre, Marie‐Hélène ; Jullien, Vincent ; Moras, Martina ; Hattab, Claude ; Lefebvre, Thibaud ; Gouya, Laurent ; Ostuni, Mariano A. ; Lefevre, Sophie D. ; Le Van Kim, Caroline</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4546-a761cd3fff93d0687fb32540756482979deb5da54e658b6c6a76ab4d15745a503</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Bilirubin</topic><topic>Erythrocytes</topic><topic>Hematology</topic><topic>Hemoglobin</topic><topic>Hsp90 protein</topic><topic>Mitochondria</topic><topic>Mitophagy</topic><topic>Oxidative stress</topic><topic>PINK1/NIX</topic><topic>PTEN-induced putative kinase</topic><topic>Sickle cell anemia</topic><topic>Sickle cell disease</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Martino, Suella</creatorcontrib><creatorcontrib>Arlet, Jean‐Benoit</creatorcontrib><creatorcontrib>Odièvre, Marie‐Hélène</creatorcontrib><creatorcontrib>Jullien, Vincent</creatorcontrib><creatorcontrib>Moras, Martina</creatorcontrib><creatorcontrib>Hattab, Claude</creatorcontrib><creatorcontrib>Lefebvre, Thibaud</creatorcontrib><creatorcontrib>Gouya, Laurent</creatorcontrib><creatorcontrib>Ostuni, Mariano A.</creatorcontrib><creatorcontrib>Lefevre, Sophie D.</creatorcontrib><creatorcontrib>Le Van Kim, Caroline</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>British journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Martino, Suella</au><au>Arlet, Jean‐Benoit</au><au>Odièvre, Marie‐Hélène</au><au>Jullien, Vincent</au><au>Moras, Martina</au><au>Hattab, Claude</au><au>Lefebvre, Thibaud</au><au>Gouya, Laurent</au><au>Ostuni, Mariano A.</au><au>Lefevre, Sophie D.</au><au>Le Van Kim, Caroline</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Deficient mitophagy pathways in sickle cell disease</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>2021-06</date><risdate>2021</risdate><volume>193</volume><issue>5</issue><spage>988</spage><epage>993</epage><pages>988-993</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><abstract>Summary
Sickle cell disease (SCD) is characterised by chronic haemolysis and oxidative stress. Herein, we investigated 30 SCD patients and found 40% with elevated mitochondria levels (SS‐mito+) in their mature red blood cells, while 60% exhibit similar mitochondria levels compared to the AA group (SS‐mito−). The SS‐mito+ patients are characterised by higher reticulocytosis and total bilirubin levels, lower foetal haemoglobin, and non‐functional mitochondria. Interestingly, we demonstrated decreased levels of mitophagy inducers, PINK1 and NIX, and higher levels of HSP90 chaperone in their red cells. Our results highlighted for the first time an abnormal retention of mitochondria in SCD linked with mitophagy‐related proteins.</abstract><cop>England</cop><pub>Blackwell Publishing Ltd</pub><pmid>33754349</pmid><doi>10.1111/bjh.17416</doi><tpages>6</tpages><orcidid>https://orcid.org/0000-0002-7504-1881</orcidid><orcidid>https://orcid.org/0000-0001-6769-3786</orcidid><orcidid>https://orcid.org/0000-0002-3251-1310</orcidid><oa>free_for_read</oa></addata></record> |
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| subjects | Bilirubin Erythrocytes Hematology Hemoglobin Hsp90 protein Mitochondria Mitophagy Oxidative stress PINK1/NIX PTEN-induced putative kinase Sickle cell anemia Sickle cell disease |
| title | Deficient mitophagy pathways in sickle cell disease |
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