Identification of the Molecular Genetic Defect of Patients With Methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an Electrostatic Model of αM Hemoglobin Assembly
We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-globin variant. Our evidence suggests that the greater-than-expected proportio...
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| Veröffentlicht in: | Blood 1999-09, Vol.94 (5), p.1825-1826 |
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| container_end_page | 1826 |
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| container_issue | 5 |
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| container_title | Blood |
| container_volume | 94 |
| creator | Ameri, A. Fairbanks, V.F. Yanik, G.A. Mahdi, F. Thibodeau, S.N. McCormick, D.J. Boxer, L.A. McDonagh, K.T. |
| description | We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. The proportion of Hb M observed is higher than that predicted for an α1-globin variant. Our evidence suggests that the greater-than-expected proportion of Hb M-Kankakee results from preferential association of the electronegative β-globin chains with the αM-globin chains that are more electropositive than normal α-globin chains. |
| doi_str_mv | 10.1182/blood.V94.5.1825 |
| format | Article |
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The proportion of Hb M observed is higher than that predicted for an α1-globin variant. Our evidence suggests that the greater-than-expected proportion of Hb M-Kankakee results from preferential association of the electronegative β-globin chains with the αM-globin chains that are more electropositive than normal α-globin chains.</description><identifier>ISSN: 0006-4971</identifier><identifier>EISSN: 1528-0020</identifier><identifier>DOI: 10.1182/blood.V94.5.1825</identifier><language>eng</language><publisher>Washington, DC: Elsevier Inc</publisher><subject>Anemias. 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Hemoglobinopathies</topic><topic>Biological and medical sciences</topic><topic>Diseases of red blood cells</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Medical sciences</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ameri, A.</creatorcontrib><creatorcontrib>Fairbanks, V.F.</creatorcontrib><creatorcontrib>Yanik, G.A.</creatorcontrib><creatorcontrib>Mahdi, F.</creatorcontrib><creatorcontrib>Thibodeau, S.N.</creatorcontrib><creatorcontrib>McCormick, D.J.</creatorcontrib><creatorcontrib>Boxer, L.A.</creatorcontrib><creatorcontrib>McDonagh, K.T.</creatorcontrib><collection>ScienceDirect Open Access Titles</collection><collection>Elsevier:ScienceDirect:Open Access</collection><collection>Pascal-Francis</collection><jtitle>Blood</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ameri, A.</au><au>Fairbanks, V.F.</au><au>Yanik, G.A.</au><au>Mahdi, F.</au><au>Thibodeau, S.N.</au><au>McCormick, D.J.</au><au>Boxer, L.A.</au><au>McDonagh, K.T.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Identification of the Molecular Genetic Defect of Patients With Methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an Electrostatic Model of αM Hemoglobin Assembly</atitle><jtitle>Blood</jtitle><date>1999-09-01</date><risdate>1999</risdate><volume>94</volume><issue>5</issue><spage>1825</spage><epage>1826</epage><pages>1825-1826</pages><issn>0006-4971</issn><eissn>1528-0020</eissn><abstract>We determined that the molecular defect of 2 patients with hemoglobin (Hb) M-Kankakee [Hb M-Iwate, α87 (F8) His → Tyr] resides in the α1-globin gene. 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| fulltext | fulltext |
| identifier | ISSN: 0006-4971 |
| ispartof | Blood, 1999-09, Vol.94 (5), p.1825-1826 |
| issn | 0006-4971 1528-0020 |
| language | eng |
| recordid | cdi_pascalfrancis_primary_1958832 |
| source | Free E-Journal (出版社公開部分のみ）; Alma/SFX Local Collection |
| subjects | Anemias. Hemoglobinopathies Biological and medical sciences Diseases of red blood cells Hematologic and hematopoietic diseases Medical sciences |
| title | Identification of the Molecular Genetic Defect of Patients With Methemoglobin M Kankakee (M-Iwate), α87 (F8) His → Tyr: Evidence for an Electrostatic Model of αM Hemoglobin Assembly |
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