A study of membrane protein defects and alpha hemoglobin chains of red blood cells in human beta thalassemia

Journal Article · Wed Nov 15 00:00:00 EST 1989 · Journal of Biological Chemistry; (USA)

OSTI ID:5023616

Rouyer-Fessard, P; Garel, M C; Domenget, C; Guetarni, D; Bachir, D; Colonna, P; Beuzard, Y ^[1]

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1. Institut National de la Sante et de la Recherche Medicale, Creteil (France)

The soluble pool of alpha hemoglobin chains present in blood or bone marrow cells was measured with a new affinity method using a specific probe, beta A hemoglobin chain labeled with ({sup 3}H)N-ethylmaleimide. This pool of soluble alpha chains was 0.067 {plus minus} 0.017% of hemoglobin in blood of normal adult, 0.11 {plus minus} 0.03% in heterozygous beta thalassemia and ranged from 0.26 to 1.30% in homozygous beta thalassemia intermedia. This elevated pool of soluble alpha chains observed in human beta thalassemia intermedia decreased 33-fold from a value of 10% of total hemoglobin in bone marrow cells to 0.3% in the most dense red blood cells. The amount of insoluble alpha chains was measured by using the polyacrylamide gel electrophoresis in urea and Triton X-100. In beta thalassemia intermedia the amount of insoluble alpha chains was correlated with the decreased spectrin content of red cell membrane and was associated with a decrease in ankyrin and with other abnormalities of the electrophoretic pattern of membrane proteins. The loss and topology of the reactive thiol groups of membrane proteins was determined by using ({sup 3}H)N-ethylmaleimide added to membrane ghosts prior to urea and Triton X-100 electrophoresis. Spectrin and ankyrin were the major proteins with the most important decrease of thiol groups.

• OSTI ID: 5023616
• Journal Information: Journal of Biological Chemistry; (USA), Vol. 264:32; ISSN 0021-9258
• Country of Publication: United States
• Language: English

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
MEMBRANE PROTEINS
BIOASSAY
THALASSEMIA
PATHOGENESIS
BONE MARROW CELLS
CELL MEMBRANES
ELECTROPHORESIS
ERYTHROCYTES
HEMOGLOBIN
IMIDES
MAN
TRACER TECHNIQUES
TRITIUM COMPOUNDS
ANEMIAS
ANIMAL CELLS
ANIMALS
BIOLOGICAL MATERIALS
BLOOD
BLOOD CELLS
BODY FLUIDS
CARBOXYLIC ACIDS
CELL CONSTITUENTS
CONNECTIVE TISSUE CELLS
DISEASES
GLOBINS
HEMIC DISEASES
HETEROCYCLIC ACIDS
HETEROCYCLIC COMPOUNDS
HYDROGEN COMPOUNDS
ISOTOPE APPLICATIONS
MAMMALS
MATERIALS
MEMBRANES
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANIC NITROGEN COMPOUNDS
PIGMENTS
PORPHYRINS
PRIMATES
PROTEINS
SOMATIC CELLS
SYMPTOMS
VERTEBRATES
550901* - Pathology- Tracer Techniques