Literature DB >> 6932957

Energy-dispersive X-ray analysis of the mitochondria of sideroblastic anaemia.

J A Grasso, T J Myers, J D Hines, A L Sullivan.   

Abstract

Energy-dispersive X-ray analysis has been performed on marrow sideroblasts obtained from 10 patients with sideroblastic anaemias or erythroleukaemia (six primary refractory sideroblastic anaemia, two pyridoxine-responsive, one secondary sideroblastic anaemia, two erythroleukaemia). Irrespective of the nature of the disorder associated with the presence of sideroblasts, X-ray analysis of siderotic mitochondria consistently revealed the presence of iron and phosphorus with the average Fe/P intensity ratio measuring 1.4-1.5. Other elements variably detected within siderotic mitochondria included calcium, lead, potassium and zinc. Variation in the presence of these latter elements was detected not only between different patients, but also within different samples taken at different times from a single patient and even among different cells of the same sample. Despite the detection of lead in siderotic mitochondria of a significant number of patients (five out of seven), there was no clinical evidence of lead toxicity. The elemental composition of the intramitochondrial deposits in sideroblasts was distinct from that of ferritin or haemosiderin and probably consists of ferric phosphate, possibly, ferric orthophosphate (FePO4).

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Year:  1980        PMID: 6932957     DOI: 10.1111/j.1365-2141.1980.tb05935.x

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


  7 in total

1.  Direct observation of iron-induced conformational changes of mitochondrial DNA by high-resolution field-emission in-lens scanning electron microscopy.

Authors:  M Yaffee; P Walter; C Richter; M Müller
Journal:  Proc Natl Acad Sci U S A       Date:  1996-05-28       Impact factor: 11.205

Review 2.  Iron metabolism in erythroid cells and patients with congenital sideroblastic anemia.

Authors:  Kazumichi Furuyama; Kiriko Kaneko
Journal:  Int J Hematol       Date:  2017-11-14       Impact factor: 2.490

3.  Distinct iron architecture in SF3B1-mutant myelodysplastic syndrome patients is linked to an SLC25A37 splice variant with a retained intron.

Authors:  V Visconte; N Avishai; R Mahfouz; A Tabarroki; J Cowen; R Sharghi-Moshtaghin; M Hitomi; H J Rogers; E Hasrouni; J Phillips; M A Sekeres; A H Heuer; Y Saunthararajah; J Barnard; R V Tiu
Journal:  Leukemia       Date:  2014-05-23       Impact factor: 11.528

4.  Combined silver Perls's stain for differential staining of ringed sideroblasts and marrow iron.

Authors:  K T Tham; J B Cousar
Journal:  J Clin Pathol       Date:  1993-08       Impact factor: 3.411

5.  SF3B1 haploinsufficiency leads to formation of ring sideroblasts in myelodysplastic syndromes.

Authors:  Valeria Visconte; Heesun J Rogers; Jarnail Singh; John Barnard; Manoj Bupathi; Fabiola Traina; James McMahon; Hideki Makishima; Hadrian Szpurka; Anna Jankowska; Andres Jerez; Mikkael A Sekeres; Yogen Saunthararajah; Anjali S Advani; Edward Copelan; Haruhiko Koseki; Kyoichi Isono; Richard A Padgett; Sami Osman; Kazunori Koide; Christine O'Keefe; Jaroslaw P Maciejewski; Ramon V Tiu
Journal:  Blood       Date:  2012-07-23       Impact factor: 22.113

6.  Identification of nonferritin mitochondrial iron deposits in a mouse model of Friedreich ataxia.

Authors:  Megan Whitnall; Yohan Suryo Rahmanto; Michael L-H Huang; Federica Saletta; Hiu Chuen Lok; Lucía Gutiérrez; Francisco J Lázaro; Adam J Fleming; Tim G St Pierre; Marc R Mikhael; Prem Ponka; Des R Richardson
Journal:  Proc Natl Acad Sci U S A       Date:  2012-11-20       Impact factor: 11.205

7.  Sideroblastic anaemia. A review of seven paediatric cases.

Authors:  B C Hamel; E D Schretlen
Journal:  Eur J Pediatr       Date:  1982-03       Impact factor: 3.183

  7 in total

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