{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T00:10:15Z","timestamp":1760832615634,"version":"build-2065373602"},"reference-count":23,"publisher":"BMJ","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Med Genet"],"published-print":{"date-parts":[[1986,6]]},"abstract":"<jats:p>A similar imbalance of globin chain synthesis, with low non-alpha\/alpha ratios, was shown in peripheral blood and in bone marrow of compound heterozygotes for both the Hb S and beta zero thalassaemia genes (S\/beta zero thalassaemia). Previous purification of whole cell globin obtained from the bone marrow did not change the non-alpha\/alpha ratio. The mean non-alpha\/alpha ratios were 0.57 +\/- 0.13 (means +\/- SD) for the peripheral blood of 12 patients, 0.52 +\/- 0.10 for five patients using bone marrow globin purified on Sephadex G100, and 0.55 +\/- 0.16 for the unfiltered bone marrow globin of five patients. The data show that patients with S\/beta zero thalassaemia have a similar beta chain deficiency in reticulocytes and in bone marrow cells, provided whole cell globin is used which avoids the removal of the free alpha chains. The non-alpha\/alpha ratios in the peripheral blood of an S\/beta zero thalassaemia patient and a beta thalassaemia heterozygote from the same family were compared in seven families and no significant difference was found.<\/jats:p>","DOI":"10.1136\/jmg.23.3.252","type":"journal-article","created":{"date-parts":[[2008,11,10]],"date-time":"2008-11-10T15:09:16Z","timestamp":1226329756000},"page":"252-255","source":"Crossref","is-referenced-by-count":1,"title":["Bone marrow and peripheral blood globin chain synthesis in sickle cell beta zero thalassaemia."],"prefix":"10.1136","volume":"23","author":[{"given":"F F","family":"Costa","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M A","family":"Zago","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[1986,6,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1111\/j.1365-2141.1973.tb05723.x","article-title":"The clinical features of sickle cell\/4-thalassemia in Jamaica","volume":"24","author":"Serjeant, G.R.; Ashcroft, M.T.; Serjeant, B.; Milner, P.F.","year":"1973","unstructured":"Serjeant GR, Ashcroft MT, Serjeant B, Milner PF. The clinical features of sickle cell\/4-thalassemia in Jamaica. Br J Haematol 1973;24: 19-30.","journal-title":"Br J Haematol"},{"key":"ref_2","article-title":"The thalassaemia syndromes","author":"Weatherall, D.J.; Clegg, J.B.","year":"1981","unstructured":"Weatherall DJ, Clegg JB. The thalassaemia syndromes. 3rd ed. Oxford: Blackwell Scientific Publications, 1981.","edition":"3rd ed"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1111\/j.1399-0004.1980.tb01366.x","article-title":"Clinical, hematological and genetic features of sickle cell-1 thalassemia in a Brazilian population","volume":"18","author":"Zago, M.A.; Costa, F.F.; Freitas, T.C.; Bottura, C.","year":"1980","unstructured":"Zago MA, Costa FF, Freitas TC, Bottura C. Clinical, hematological and genetic features of sickle cell-1 thalassemia in a Brazilian population. 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