Stomatin is mis-trafficked in the erythrocytes of overhydrated hereditary stomatocytosis, and is absent from normal primitive yolk sac-derived erythrocytes.

Abstract:

:The 32 kD lipid-raft-associated membrane protein 'stomatin' is deficient from the erythrocyte membrane in the Na+-K+ leaky haemolytic anaemia, overhydrated hereditary stomatocytosis (OHSt). To date, no mutation in the gene coding for this protein has so far been found in OHSt. In this study, we have analysed the distribution of stomatin in both cultured erythroid cells from OHSt patients and in normal embryological and fetal erythroid development. In erythroid cell cultures from OHSt patients, stomatin-immunoreactivity (stomatin-IR) was present in progenitor cells but remained restricted to the area of the multivesicular complexes and the nucleus in the developing cells and was not seen in the plasma membrane. This could be consistent with the idea that stomatin is an innocent passenger in a more fundamental trafficking abnormality. In normal embryonic development, we found that, in extraembryonic (yolk sac) erythropoiesis, neither the nucleated red cells nor their enucleated mature derivatives displayed any stomatin-IR. In contrast, all haemangiopoietic progenitor cells of intraembryonic haematopoiesis, starting with the mesodermal precursors in the aorta-gonad-mesonephros region, exhibited strong stomatin-IR. The significance of this observation on these poorly understood cells is currently unclear.

journal_name

Br J Haematol

authors

Fricke B,Parsons SF,Knöpfle G,von Düring M,Stewart GW

doi

10.1111/j.1365-2141.2005.05742.x

subject

Has Abstract

pub_date

2005-10-01 00:00:00

pages

265-77

issue

2

eissn

0007-1048

issn

1365-2141

pii

BJH5742

journal_volume

131

pub_type

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