{"id":"https://openalex.org/W2502804636","doi":"https://doi.org/10.1182/blood.v114.22.4065.4065","title":"Heightened Sulfur Amino Acid Oxidation in Plasma and Erythrocytes in \u03b2-Thalassemia Major.","display_name":"Heightened Sulfur Amino Acid Oxidation in Plasma and Erythrocytes in \u03b2-Thalassemia Major.","publication_year":2009,"publication_date":"2009-11-20","ids":{"openalex":"https://openalex.org/W2502804636","doi":"https://doi.org/10.1182/blood.v114.22.4065.4065","mag":"2502804636"},"language":"en","primary_location":{"id":"doi:10.1182/blood.v114.22.4065.4065","doi":"https://doi.org/10.1182/blood.v114.22.4065.4065","is_oa":false,"landing_page_url":"https://doi.org/10.1182/blood.v114.22.4065.4065","pdf_url":null,"source":{"id":"https://openalex.org/S200071133","display_name":"Blood","issn_l":"0006-4971","issn":["0006-4971","1528-0020"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Blood","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5007078872","display_name":"Anurag K. Agrawal","orcid":"https://orcid.org/0000-0003-4898-9615","observed_orcids":["https://orcid.org/0000-0003-4898-9615"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Anurag K. Agrawal","raw_affiliation_strings":["Pediatric Hematology/Oncology, Children's Hospital and Research Center, Oakland, CA, USA,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pediatric Hematology/Oncology, Children's Hospital and Research Center, Oakland, CA, USA,","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5113733144","display_name":"Jung H. Suh","orcid":"https://orcid.org/0000-0001-8119-326X","observed_orcids":["https://orcid.org/0000-0001-8119-326X"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Jung H. Suh","raw_affiliation_strings":["Nutrition and Metabolism Center, Children's Hospital Oakland Research Institute, Oakland, CA, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nutrition and Metabolism Center, Children's Hospital Oakland Research Institute, Oakland, CA, USA","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5110195895","display_name":"Bruce N. Ames","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Bruce N. Ames","raw_affiliation_strings":["Nutrition and Metabolism Center, Children's Hospital Oakland Research Institute, Oakland, CA, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Nutrition and Metabolism Center, Children's Hospital Oakland Research Institute, Oakland, CA, USA","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5056946591","display_name":"Elliott P. Vichinsky","orcid":"https://orcid.org/0000-0002-0500-9579","observed_orcids":["https://orcid.org/0000-0002-0500-9579"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Elliott P. Vichinsky","raw_affiliation_strings":["Pediatric Hematology/Oncology, Children's Hospital and Research Center, Oakland, CA, USA,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pediatric Hematology/Oncology, Children's Hospital and Research Center, Oakland, CA, USA,","institution_ids":[]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5005668427","display_name":"Ashutosh Lal","orcid":"https://orcid.org/0000-0003-0082-3536","observed_orcids":["https://orcid.org/0000-0003-0082-3536"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Ashutosh Lal","raw_affiliation_strings":["Pediatric Hematology/Oncology, Children's Hospital and Research Center, Oakland, CA, USA,"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pediatric Hematology/Oncology, Children's Hospital and Research Center, Oakland, CA, USA,","institution_ids":[]}]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":["https://openalex.org/A5007078872","https://openalex.org/A5110195895","https://openalex.org/A5113733144"],"corresponding_institution_ids":[],"apc_list":{"value":5500,"currency":"USD","value_usd":5500},"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.34830259,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"114","issue":"22","first_page":"4065","last_page":"4065"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10554","display_name":"Hemoglobinopathies and Related Disorders","score":0.9980000257492065,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10554","display_name":"Hemoglobinopathies and Related Disorders","score":0.9980000257492065,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11481","display_name":"Sulfur Compounds in Biology","score":0.0003000000142492354,"subfield":{"id":"https://openalex.org/subfields/1303","display_name":"Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10445","display_name":"Antioxidant Activity and Oxidative Stress","score":0.0003000000142492354,"subfield":{"id":"https://openalex.org/subfields/2704","display_name":"Biochemistry (medical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/beta-thalassemia-major","display_name":"beta-thalassemia major","score":0.9980000257492065},{"id":"https://openalex.org/keywords/oxidative-stress","display_name":"oxidative stress","score":0.8690000176429749},{"id":"https://openalex.org/keywords/glutathione","display_name":"glutathione","score":0.9169999957084656},{"id":"https://openalex.org/keywords/cysteine","display_name":"cysteine","score":0.843999981880188},{"id":"https://openalex.org/keywords/erythrocytes","display_name":"erythrocytes","score":0.7770000100135803},{"id":"https://openalex.org/keywords/iron-overload","display_name":"iron overload","score":0.7670000195503235},{"id":"https://openalex.org/keywords/blood-transfusion","display_name":"blood transfusion","score":0.4519999921321869},{"id":"https://openalex.org/keywords/antioxidant-defense","display_name":"antioxidant defense","score":0.7210000157356262},{"id":"https://openalex.org/keywords/liver-iron-concentration","display_name":"liver iron concentration","score":0.49399998784065247},{"id":"https://openalex.org/keywords/cardiac-iron-overload","display_name":"cardiac iron overload","score":0.8389999866485596},{"id":"https://openalex.org/keywords/t2","display_name":"T2*","score":0.36800000071525574}],"concepts":[{"id":"https://openalex.org/C538909803","wikidata":"https://www.wikidata.org/wiki/Q116907","display_name":"Glutathione","level":3,"score":0.7007685899734497},{"id":"https://openalex.org/C2776151105","wikidata":"https://www.wikidata.org/wiki/Q898814","display_name":"Oxidative stress","level":2,"score":0.6989243626594543},{"id":"https://openalex.org/C2777799968","wikidata":"https://www.wikidata.org/wiki/Q185137","display_name":"Thalassemia","level":2,"score":0.6823294162750244},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.5775151252746582},{"id":"https://openalex.org/C2778004101","wikidata":"https://www.wikidata.org/wiki/Q133948","display_name":"Antioxidant","level":2,"score":0.5202304720878601},{"id":"https://openalex.org/C2776557347","wikidata":"https://www.wikidata.org/wiki/Q37187","display_name":"Red blood cell","level":2,"score":0.5009725093841553},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.4960709512233734},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.3972422182559967},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.37337255477905273},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.33717840909957886},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1182/blood.v114.22.4065.4065","doi":"https://doi.org/10.1182/blood.v114.22.4065.4065","is_oa":false,"landing_page_url":"https://doi.org/10.1182/blood.v114.22.4065.4065","pdf_url":null,"source":{"id":"https://openalex.org/S200071133","display_name":"Blood","issn_l":"0006-4971","issn":["0006-4971","1528-0020"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Blood","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":0.9537000060081482}],"study_designs":[{"display_name":"Observational Study","id":"https://openalex.org/study-designs/observational-study"}],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W4288213198","https://openalex.org/W1606615931","https://openalex.org/W2146489763","https://openalex.org/W2187997178","https://openalex.org/W2189502811","https://openalex.org/W70287413","https://openalex.org/W7217086242","https://openalex.org/W2910107403","https://openalex.org/W3110998861","https://openalex.org/W4405388633"],"abstract_inverted_index":{"Abstract":[0,1],"4065":[2],"Poster":[3],"Board":[4],"III-1000":[5],"Background":[6],"Oxidative":[7],"stress":[8,68,408,533],"in":[9,18,61,69,106,215,224,245,252,268,275,286,300,313,323,332,352,358,368,431,436,446,462,482,488,509,524,535,563],"\u03b2-thalassemia":[10,121,164,432],"major":[11,102,122,165],"is":[12],"a":[13,73,320,427,452,460],"well":[14,97],"documented":[15],"problem":[16],"thought":[17],"part":[19],"to":[20,23,43,51,65,85,132,248,271,344,356,425,556,571],"be":[21,350,426,474,550,561],"due":[22],"transfusional":[24],"iron":[25,182,208,387,394,540],"overload.":[26],"Sulfur":[27],"amino":[28,103],"acid":[29],"(SAA)-derived":[30],"metabolites,":[31],"chiefly":[32],"glutathione":[33,411],"(GSH),":[34],"are":[35],"critical":[36],"components":[37],"of":[38,54,91,101,171,206,217,230,306,347,386,406,415,456,468,479,519,530,569],"cellular":[39],"antioxidant":[40,47],"defense.":[41],"Inability":[42],"adequately":[44],"regulate":[45],"endogenous":[46],"status":[48,237],"may":[49,349,473,548],"lead":[50],"further":[52],"exacerbation":[53],"oxidative":[55,67,407,507,532],"stress.":[56],"To":[57],"characterize":[58],"whether":[59],"alteration":[60],"SAA":[62,221,341,359,416,457],"metabolism":[63],"contributes":[64],"heightened":[66],"thalassemia,":[70,483],"we":[71,503],"utilized":[72],"novel":[74,428],"liquid":[75],"chromatography":[76],"linked":[77],"electrospray":[78],"positive":[79],"tandem":[80],"mass":[81],"spectrometric":[82],"(LC-MS/MS)":[83],"technique":[84,149],"simultaneously":[86],"quantify":[87],"the":[88,99,133,147,172,199,256,287,293,297,301,304,362,384,404,423,463,477,506,510,513,525,557],"redox":[89,222,236,259,417,441],"states":[90,418,442],"cysteine":[92],"(Cys)":[93],"and":[94,108,117,139,155,232,282,335,372,396,419,439],"GSH":[95,258,281,326,348,440],"as":[96,98],"concentrations":[100],"acid-derived":[104],"metabolites":[105,522],"plasma":[107,257,288,302,333,369,511,526],"erythrocytes.":[109],"Methods":[110],"After":[111],"institutional":[112],"review":[113],"board":[114],"approval,":[115],"adult":[116],"pediatric":[118],"subjects":[119,162,226,277,489,536],"with":[120,157,163,375,403,490,537],"were":[123,144,166],"recruited":[124],"for":[125,198,465],"fasting":[126],"blood":[127,136,141],"samples":[128,143],"drawn":[129],"immediately":[130],"prior":[131],"next":[134],"scheduled":[135],"transfusion.":[137],"Plasma":[138,220],"red":[140],"cell":[142],"analyzed":[145],"using":[146],"LC-MS/MS":[148],"(J":[150],"Chromatogr":[151],"B":[152],"2009;in":[153],"press)":[154],"compared":[156,374],"healthy":[158,246,269],"controls.":[159,376],"Results":[160],"Twelve":[161],"recruited.":[167],"The":[168,179,194,279,434,471,528],"median":[169,180,195],"age":[170],"group":[173,200,364],"was":[174,188,201,213,309,365],"26.5":[175],"years":[176],"(range":[177,192],"11-41).":[178],"liver":[181,393],"concentration":[183,395,449],"(LIC)":[184],"determined":[185],"by":[186,390,410,476],"ferritometer":[187],"11.9":[189],"mg/g":[190],"dry-wt":[191],"1.0-34.8).":[193],"ferritin":[196],"level":[197,385],"1980":[202],"ng/mL":[203],"(379-4730).":[204],"Evidence":[205],"myocardial":[207],"overload":[209,388],"(T2*":[210],"<20":[211],"msec)":[212],"present":[214],"5":[216],"11":[218],"subjects.":[219],"analysis":[223],"these":[225],"showed":[227,400],"significant":[228,329,453,531],"oxidation":[229,458],"Cys":[231,235,284,438],"GSH.":[233],"Total":[234],"(Cysteine/2*Cystine":[238],"ratios)":[239],"decreased":[240,263],"from":[241,264],"0.1":[242],"\u00b1":[243,250,266,273],"0.01":[244],"controls":[247,270],"0.08":[249],"0.02":[251],"thalassemia":[253,276,314,363],"(p=0.02).":[254],"Similarly,":[255],"state":[260,559],"(GSH/2*GSSG":[261],"ratio)":[262],"38.9":[265],"13.7":[267],"6.7":[272],"5.3":[274],"(p=0.005).":[278],"total":[280,283,324,448],"pools":[285],"did":[289],"not":[290,549],"differ":[291],"between":[292],"two":[294],"groups.":[295],"Mirroring":[296],"patterns":[298],"observed":[299,523],"compartment,":[303],"proportion":[305],"erythrocyte":[307,325],"GSSG":[308],"also":[310],"significantly":[311],"elevated":[312],"(0.65%":[315],"vs.":[316],"0.04%,":[317],"p=0.009),":[318],"without":[319,443],"concomitant":[321,444],"decline":[322,445,461],"pool.":[327],"A":[328,500],"3-fold":[330],"elevation":[331],"cystathionine":[334],"S-adenosylmethionine":[336],"levels":[337,547],"suggests":[338,450,542],"that":[339,379,505,543,553],"key":[340],"metabolic":[342,420],"pathways":[343,421],"augment":[345],"synthesis":[346],"up-regulated":[351],"thalassemia.":[353,564],"In":[354],"addition":[355],"alterations":[357,435],"metabolite":[360],"profiles,":[361],">3-fold":[366],"deficient":[367],"valine,":[370],"spermine":[371],"citrulline":[373],"We":[377],"found":[378],"within":[380],"this":[381],"small":[382],"group,":[383],"measured":[389,409],"serum":[391],"ferritin,":[392],"cardiac":[397],"MRI":[398],"T2*":[399],"no":[401],"correlation":[402],"amount":[405],"redox.":[412],"Discussion":[413],"Assessment":[414],"has":[422,485],"potential":[424],"important":[429],"marker":[430],"major.":[433],"both":[437],"their":[447],"either":[451],"increased":[454],"rate":[455],"and/or":[459],"capacity":[464],"NADPH-dependent":[466],"reduction":[467],"oxidized":[469],"disulfides.":[470],"latter":[472],"supported":[475],"evidence":[478],"profound":[480],"hypocitrullinemia":[481],"which":[484,516],"been":[486],"noted":[487],"inherited":[491],"mitochondrial":[492],"disorders":[493],"(Proc":[494],"Nat":[495],"Acad":[496],"Sci":[497],"U":[498],"S":[499],"2009;106:3941-5).":[501],"Finally,":[502],"show":[504],"environment":[508],"affects":[512],"transfused":[514],"erythrocytes":[515],"acquire":[517],"characteristics":[518],"abnormal":[520],"thiol":[521],"compartment.":[527],"demonstration":[529],"even":[534],"low":[538],"body":[539],"burden":[541],"currently":[544],"acceptable":[545],"LIC":[546],"optimal,":[551],"or":[552],"other":[554],"contributors":[555],"pro-oxidant":[558],"should":[560],"sought":[562],"Disclosures:":[565],"No":[566],"relevant":[567],"conflicts":[568],"interest":[570],"declare.":[572]},"counts_by_year":[],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-08-23T00:00:00"}