[{"@context":"http:\/\/schema.org\/","@type":"BlogPosting","@id":"https:\/\/wiki.edu.vn\/en\/hemoglobinopathy-wikipedia\/#BlogPosting","mainEntityOfPage":"https:\/\/wiki.edu.vn\/en\/hemoglobinopathy-wikipedia\/","headline":"Hemoglobinopathy – Wikipedia","name":"Hemoglobinopathy – Wikipedia","description":"before-content-x4 From Wikipedia, the free encyclopedia after-content-x4 Medical condition Hemoglobinopathy is the medical term for a group of inherited blood","datePublished":"2019-09-27","dateModified":"2019-09-27","author":{"@type":"Person","@id":"https:\/\/wiki.edu.vn\/en\/author\/lordneo\/#Person","name":"lordneo","url":"https:\/\/wiki.edu.vn\/en\/author\/lordneo\/","image":{"@type":"ImageObject","@id":"https:\/\/secure.gravatar.com\/avatar\/cd810e53c1408c38cc766bc14e7ce26a?s=96&d=mm&r=g","url":"https:\/\/secure.gravatar.com\/avatar\/cd810e53c1408c38cc766bc14e7ce26a?s=96&d=mm&r=g","height":96,"width":96}},"publisher":{"@type":"Organization","name":"Enzyklop\u00e4die","logo":{"@type":"ImageObject","@id":"https:\/\/wiki.edu.vn\/wiki4\/wp-content\/uploads\/2023\/08\/download.jpg","url":"https:\/\/wiki.edu.vn\/wiki4\/wp-content\/uploads\/2023\/08\/download.jpg","width":600,"height":60}},"image":{"@type":"ImageObject","@id":"https:\/\/upload.wikimedia.org\/wikipedia\/commons\/thumb\/a\/a0\/B-Thal.jpg\/220px-B-Thal.jpg","url":"https:\/\/upload.wikimedia.org\/wikipedia\/commons\/thumb\/a\/a0\/B-Thal.jpg\/220px-B-Thal.jpg","height":"147","width":"220"},"url":"https:\/\/wiki.edu.vn\/en\/hemoglobinopathy-wikipedia\/","wordCount":4618,"articleBody":" (adsbygoogle = window.adsbygoogle || []).push({});before-content-x4From Wikipedia, the free encyclopedia (adsbygoogle = window.adsbygoogle || []).push({});after-content-x4Medical conditionHemoglobinopathy is the medical term for a group of inherited blood disorders and diseases that primarily affect red blood cells.[1] They are single-gene disorders and, in most cases, they are inherited as autosomal co-dominant traits.[2] (adsbygoogle = window.adsbygoogle || []).push({});after-content-x4There are two main groups: abnormal structural hemoglobin variants caused by mutations in the hemoglobin genes, and the thalassemias, which are caused by an underproduction of otherwise normal hemoglobin molecules. The main structural hemoglobin variants are HbS, HbE and HbC. The main types of thalassemia are alpha-thalassemia and beta thalassemia.[3]The two conditions may overlap because some conditions which cause abnormalities in hemoglobin proteins also affect their production. Some hemoglobin variants do not cause pathology or anemia, and thus are often not classed as hemoglobinopathies.[4][5]Table of Contents (adsbygoogle = window.adsbygoogle || []).push({});after-content-x4Hemoglobin structural biology[edit]Classification of hemoglobinopathies[edit]A) Qualitative[edit]Structural abnormalities[edit]Chemical abnormalities[edit]B) Quantitative[edit]Production abnormalities[edit]Hemoglobin variants[edit]Electrophoretic migration patterns[edit]Alkaline electrophoresis[edit]Acid electrophoresis[edit]Evolution[edit]References[edit]External links[edit]Hemoglobin structural biology[edit]Normal human hemoglobins are tetrameric proteins composed of two pairs of globin chains, each of which contains one alpha-like (\u03b1-like) chain and one beta-like (\u03b2-like) chain. Each globin chain is associated with an iron-containing heme moiety. Throughout life, the synthesis of the alpha-like and the beta-like (also called non-alpha-like) chains is balanced so that their ratio is relatively constant and there is no excess of either type.[6]The specific alpha and beta-like chains that are incorporated into Hb are highly regulated during development:[citation needed]Embryonic Hbs are expressed as early as four to six weeks of embryogenesis and disappear around the eighth week of gestation as they are replaced by fetal Hb.[7][8] Embryonic Hbs include:Hb Gower-1, composed of two \u03b6 globins (zeta globins) and two \u03b5 globins (epsilon globins) (\u03b62\u03b52)Hb Gower-2, composed of two alpha globins and two epsilon globins (\u03b12\u03b52)Hb Portland, composed of two zeta globins and two gamma globins (\u03b62\u03b32)Fetal Hb (Hb F) is produced from approximately eight weeks of gestation through birth and constitutes approximately 80 percent of Hb in the full-term neonate. It declines during the first few months of life and, in the normal state, constitutes (adsbygoogle = window.adsbygoogle || []).push({});after-content-x4"},{"@context":"http:\/\/schema.org\/","@type":"BreadcrumbList","itemListElement":[{"@type":"ListItem","position":1,"item":{"@id":"https:\/\/wiki.edu.vn\/en\/#breadcrumbitem","name":"Enzyklop\u00e4die"}},{"@type":"ListItem","position":2,"item":{"@id":"https:\/\/wiki.edu.vn\/en\/hemoglobinopathy-wikipedia\/#breadcrumbitem","name":"Hemoglobinopathy – Wikipedia"}}]}]