Literature DB >> 1201222

Observations on the ultrastructure of erythropoietic cells and reticulum cells in the bone marrow of patients with homozygous beta-thalassaemia.

S N Wickramasinghe, V Bush.   

Abstract

An electron microscopic study of marrow fragments from patients with homozygous beta-thalassaemia has shown that 3% of early polychromatic erythroblast profiles and 20% of late polychromatic erythroblast profiles contain intracytoplasmic alpha-chain precipitates. Various nuclear abnormalities were found including the loss of parts of the nuclear membrane and the presence of intranuclear alpha-chain precipitates, and these abnormalities were virtually confined to the non-dividing, late polychromatic erythroblasts. As most profiles of the proliferating early polychromatic erythroblasts did not contain intracytoplasmic or intranuclear alpha-chain precipitates, it is suggested that the arrest of many of these cells in the G1 phase of the cell cycle may be related to the presence of an excess of free alpha-chains rather than to the presence of alpha-chain precipitates within them. The cytoplasm of the bone marrow reticulum cells contained early and late polychromatic erythroblasts at various stages of degradation, providing direct evidence of ineffective erythropoiesis.

Entities:  

Mesh:

Year:  1975        PMID: 1201222     DOI: 10.1111/j.1365-2141.1975.tb01853.x

Source DB:  PubMed          Journal:  Br J Haematol        ISSN: 0007-1048            Impact factor:   6.998


  8 in total

Review 1.  Protein quality control during erythropoiesis and hemoglobin synthesis.

Authors:  Eugene Khandros; Mitchell J Weiss
Journal:  Hematol Oncol Clin North Am       Date:  2010-12       Impact factor: 3.722

2.  Integrated protein quality-control pathways regulate free α-globin in murine β-thalassemia.

Authors:  Eugene Khandros; Christopher S Thom; Janine D'Souza; Mitchell J Weiss
Journal:  Blood       Date:  2012-03-16       Impact factor: 22.113

3.  Beta-thalassaemia: increased chromosomal anomalies in lymphocyte cultures.

Authors:  G B Côté; S Papadakou-Lagoyanni
Journal:  J Med Genet       Date:  1979-02       Impact factor: 6.318

4.  Heme-regulated eIF2alpha kinase (HRI) is required for translational regulation and survival of erythroid precursors in iron deficiency.

Authors:  A P Han; C Yu; L Lu; Y Fujiwara; C Browne; G Chin; M Fleming; P Leboulch; S H Orkin; J J Chen
Journal:  EMBO J       Date:  2001-12-03       Impact factor: 11.598

Review 5.  Redox Balance in β-Thalassemia and Sickle Cell Disease: A Love and Hate Relationship.

Authors:  Rayan Bou-Fakhredin; Lucia De Franceschi; Irene Motta; Assaad A Eid; Ali T Taher; Maria Domenica Cappellini
Journal:  Antioxidants (Basel)       Date:  2022-05-13

Review 6.  Ineffective erythropoiesis in β -thalassemia.

Authors:  Jean-Antoine Ribeil; Jean-Benoit Arlet; Michael Dussiot; Ivan Cruz Moura; Geneviève Courtois; Olivier Hermine
Journal:  ScientificWorldJournal       Date:  2013-03-28

Review 7.  Oxidative stress in β-thalassaemia and sickle cell disease.

Authors:  S Voskou; M Aslan; P Fanis; M Phylactides; M Kleanthous
Journal:  Redox Biol       Date:  2015-08-01       Impact factor: 11.799

8.  A synthetic model of human beta-thalassemia erythropoiesis using CD34+ cells from healthy adult donors.

Authors:  Y Terry Lee; Ki Soon Kim; Colleen Byrnes; Jaira F de Vasconcellos; Seung-Jae Noh; Antoinette Rabel; Emily R Meier; Jeffery L Miller
Journal:  PLoS One       Date:  2013-07-08       Impact factor: 3.240

  8 in total

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