title: A G gamma type of the hereditary persistence of fetal hemoglobin with beta chain production in cis.
abstract: In a new subclass of G gamma HPFH which has been detected in a black family, beta A chains are produced in cis to the HPFH determinant (the G gamma-beta+ HPFH). No other instance of beta chain production in cis to HPFH has been reported. All individuals in this family are well even if Hb S is produced in trans to HPFH. Genetically, this new subclass requires a slightly smaller deletion in the gamma, delta, and beta complex of genes than do other forms of HPFH. It is speculated that a subclass (the G gamma-(G gamma A gamma)-beta+ HPFH) in which beta S chains are produced in cis to HPFH in conjunction with true beta S genes in trans may be responsible for "mild" cases of sickle cell anemia.
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D004592:Electrophoresis, Starch Gel; D004906:Erythrocyte Count; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis*; D008040:Genetic Linkage; D005826:Genetics, Medical; D005914:Globins / Q000032:analysis; D006400:Hematocrit; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D013789:Thalassemia / Q000235:genetics*
chemical_list: D006454:Hemoglobins; D005914:Globins; D005319:Fetal Hemoglobin