{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,29]],"date-time":"2025-10-29T18:33:47Z","timestamp":1761762827154},"reference-count":24,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2006,7,11]],"date-time":"2006-07-11T00:00:00Z","timestamp":1152576000000},"content-version":"vor","delay-in-days":7223,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[1986,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The Sp\u03b1<jats:sup>1\/65<\/jats:sup> variant of the spectrin has been recently described in black people with hereditary elliptocytosis (HE). The present study reports on a similar Sp\u03b1<jats:sup>1\/65<\/jats:sup> variant in nine North African persons belonging to four unrelated families. The abnormality was associated with a variable degree of elliptocytosis. In one case, red cell morphology was normal. In the nine carriers of the biochemical abnormality, the spectrin dimer self\u2010association was defective. The association constant was reduced: 0.65 to 1.7 \u00d7 10<jats:sup>5<\/jats:sup> M<jats:sup>\u22121<\/jats:sup> (controls: 4.6 \u00d7 0.5 \u00d7 10<jats:sup>5<\/jats:sup> m M<jats:sup>\u22121<\/jats:sup> (n = 21)); in six cases, there was a higher level of spectrin dimer in the low ionic strength extract at 4\u00b0C: 13.0 to 19.7% (controls: 6.4 \u00b1 2.1% (n = 7)). Limited tryptic digests of spectrin from the nine persons revealed a decrease of the 80,000\u2010dalton \u03b1\u20101 domain, and the concomitant appearance of a peptide with a molecular weight of 65,000 daltons and an isoelectric point ranging from 5.0 to 5.1. There was a correlation between the proportion of the 65,000\u2010dalton fragments, the defect of spectrin self\u2010association, and the extent of morphological alteration. This is the first large series concerning a spectrin abnormality in non\u2010black persons. In North Africa, cases of HE that are not due to a protein 4.1 defect have turned out so far to be associated with the Sp\u03b1<jats:sup>1\/65<\/jats:sup> variant.<\/jats:p>","DOI":"10.1002\/ajh.2830230205","type":"journal-article","created":{"date-parts":[[2007,2,21]],"date-time":"2007-02-21T10:44:15Z","timestamp":1172054655000},"page":"113-122","source":"Crossref","is-referenced-by-count":17,"title":["Sp\u03b1<sup>1\/65<\/sup> hereditary elliptocytosis in North Africa"],"prefix":"10.1002","volume":"23","author":[{"given":"N.","family":"Alloisio","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Guetarni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L.","family":"Morl\u00e9","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Pothier","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. T.","family":"Ducluzeau","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Soun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Colonna","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Clerc","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Philippe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Delaunay","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,7,11]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/S0308-2261(21)00462-8","article-title":"Hereditary elliptocytosis and related disorders","volume":"14","author":"Palek J","year":"1985","journal-title":"Clin Haematol"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00278857"},{"key":"e_1_2_1_4_2","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1182\/blood.V65.1.46.46","article-title":"The characterization of heterozygous 4.1 (\u2010) hereditary elliptocytosis (the 4.1 (\u2010) trait)","volume":"65","author":"Alloisio N","year":"1985","journal-title":"Blood"},{"key":"e_1_2_1_5_2","volume-title":"Erythrocyte Membrane 2: Recent Clinical and Experimental Advances","author":"Mueller TJ","year":"1981"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110666"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1985.tb04057.x"},{"key":"e_1_2_1_8_2","first-page":"1005","article-title":"Hereditary elliptocytosis with a spectrin molecular defect in a white patient","volume":"18","author":"Lecomte MC","year":"1984","journal-title":"Acta Haematol"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI111376"},{"key":"e_1_2_1_10_2","doi-asserted-by":"crossref","first-page":"1208","DOI":"10.1182\/blood.V65.5.1208.1208","article-title":"A new abnormal variant of spectrin in Black patients with hereditary elliptocytosis","volume":"65","author":"Lecomte MC","year":"1985","journal-title":"Blood"},{"key":"e_1_2_1_11_2","doi-asserted-by":"crossref","first-page":"706","DOI":"10.1182\/blood.V66.3.706.706","article-title":"Sp\u03b11\/65: A new variant of the \u03b1 subunit of spectrin in hereditary elliptocytosis","volume":"66","author":"Lawler J","year":"1985","journal-title":"Blood"},{"key":"e_1_2_1_12_2","first-page":"315","article-title":"Premiere observation de l'absence d'une prot\u00e9ine de la membrane \u00e9rythrocytaire (bande 41) dans un cas d'an\u00e9mie elliptocytaire familiale","volume":"22","author":"F\u00e9o C","year":"1980","journal-title":"Nouv Rev Fr Hematol"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110275"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9861(63)90042-0"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi00803a007"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1038\/227680a0"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1432-1033.1978.tb12459.x"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi00842a023"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110293"},{"key":"e_1_2_1_20_2","doi-asserted-by":"crossref","first-page":"4007","DOI":"10.1016\/S0021-9258(19)41496-8","article-title":"High resolution two\u2010dimensional electrophoresis of proteins","volume":"251","author":"O'Farrell PH","year":"1975","journal-title":"J Biol Chem"},{"key":"e_1_2_1_21_2","doi-asserted-by":"crossref","first-page":"9093","DOI":"10.1016\/S0021-9258(18)34247-9","article-title":"A structural model of human erythrocyte spectrin","volume":"257","author":"Speicher DW","year":"1982","journal-title":"J Biol Chem"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110689"},{"key":"e_1_2_1_23_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110942"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/0378-1119(85)90191-X"},{"key":"e_1_2_1_25_2","doi-asserted-by":"crossref","first-page":"1182","DOI":"10.1182\/blood.V62.6.1182.1182","article-title":"Molecular heterogeneity of hereditary pyropoikilocytosis: Identification of a second variant of the spectrin \u03b1\u2010chain","volume":"62","author":"Lawler J","year":"1983","journal-title":"Blood"}],"container-title":["American Journal of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajh.2830230205","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajh.2830230205","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,21]],"date-time":"2023-10-21T01:09:17Z","timestamp":1697850557000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajh.2830230205"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1986,10]]},"references-count":24,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1986,10]]}},"alternative-id":["10.1002\/ajh.2830230205"],"URL":"https:\/\/doi.org\/10.1002\/ajh.2830230205","archive":["Portico"],"relation":{},"ISSN":["0361-8609","1096-8652"],"issn-type":[{"value":"0361-8609","type":"print"},{"value":"1096-8652","type":"electronic"}],"subject":[],"published":{"date-parts":[[1986,10]]}}}