{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,7]],"date-time":"2026-02-07T22:44:04Z","timestamp":1770504244627,"version":"3.49.0"},"reference-count":34,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,10,19]],"date-time":"2004-10-19T00:00:00Z","timestamp":1098144000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2004,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Hemoglobin (Hb) Bassett, an abnormal Hb variant with a markedly reduced oxygen affinity, was discovered in a Caucasian (Anglo\u2010Saxon) male child who experienced episodes of cyanosis. Cation\u2010exchange and reversed\u2010phase (RP) high\u2010performance liquid chromatography (HPLC) showed that the patient has an abnormal Hb, with a mutation in the \u03b1\u2010globin. Tryptic peptide digest of the abnormal \u03b1\u2010globin with subsequent HPLC analysis revealed abnormal elution of the \u03b1\u2010T11 peptide. Further studies with Edman sequencing and electrospray mass spectrometry of tryptic peptide \u03b1\u2010T11, as well as structural analysis by X\u2010ray crystallography revealed an Asp\u2192Ala substitution at the \u03b194 (G1) position, a match for Hb Bassett. Detailed functional studies showed that this Hb variant had a markedly reduced oxygen affinity (<jats:italic>P<\/jats:italic><jats:sub>50<\/jats:sub> at pH 7.0 = 22 mmHg; Hb A <jats:italic>P<\/jats:italic><jats:sub>50<\/jats:sub> = 10.5 mmHg), reduced Bohr effect (\u22120.26 compared to \u2212 0.54 in Hb A), and low subunit cooperativity (<jats:italic>n<\/jats:italic> = 1.4, compared to 2.6 in Hb A). X\u2010ray crystallography results explain the probable effects of the structural modification on the oxygen\u2010binding properties of this Hb variant. Am. J. Hematol. 77:268\u2013276, 2004. \u00a9 2004 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajh.20184","type":"journal-article","created":{"date-parts":[[2004,10,19]],"date-time":"2004-10-19T16:03:10Z","timestamp":1098201790000},"page":"268-276","source":"Crossref","is-referenced-by-count":26,"title":["Characterization of hemoglobin bassett (\u03b194Asp\u2192Ala), a variant with very low oxygen affinity"],"prefix":"10.1002","volume":"77","author":[{"given":"Osheiza","family":"Abdulmalik","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin K.","family":"Safo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Norma B.","family":"Lerner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Josiree","family":"Ochotorena","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Evgueni","family":"Daikhin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vinaysagar","family":"Lakka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosa","family":"Santacroce","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donald J.","family":"Abraham","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshio","family":"Asakura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,19]]},"reference":[{"key":"e_1_2_1_2_2","volume-title":"Hemoglobin: molecular, genetic and clinical aspects","author":"Bunn FH","year":"1986"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.3109\/03630269409014146"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.3109\/03630267908998910"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.3109\/03630269708997513"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/0167-4838(94)00190-R"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2795(75)90192-0"},{"issue":"5","key":"e_1_2_1_8_2","doi-asserted-by":"crossref","first-page":"980","DOI":"10.1016\/S0021-9258(18)93612-4","article-title":"Hemoglobin Kansas, a human hemoglobin with a neutral amino acid substitution and an abnormal oxygen equilibrium","volume":"243","author":"Bonaventura J","year":"1968","journal-title":"J Biol Chem"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI104406"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.3109\/03630267808999192"},{"issue":"17","key":"e_1_2_1_11_2","doi-asserted-by":"crossref","first-page":"6939","DOI":"10.1016\/S0021-9258(19)41022-3","article-title":"Hemoglobin Richmond. 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