Inborn anemias in mice: (Annual report, 1982-1983)

Technical Report · Fri Sep 09 00:00:00 EDT 1983

OSTI ID:6586627

Bernstein, S E

The nature of the defects that shorten the effective lifespan of red blood cells in the circulation and which gave rise to anemia, jaundice and to spleen, liver and heart enlargement are studied because they so closely parallel inherited hemolytic anemias in man. In mice, ''hemolytic disease'' initiated by the ja, sph, sph/sup ha/, or the nb genes has been traced to abnormalities in the protein components of their red cell membranes. Polyacrylamide gel electrophoresis of detergent solubilized membranes reveal that in the different genetic types one or more of the major high molecular weight proteins called spectrins is decreased or totally missing. It is one thing to observe a correlation between missing or defective components in selected analytical procedures, and another to establish a causal relationship between the two. To investigate the possible interrelationships, we examined the associations between spectrin or ankyrin content, the severity of the resulting anemia, red cell osmotic fragilities, and the capacity of cells from each genotype to be deformed in a continuous osmotic gradient at constant sheer stress. Our findings indicate that sensitivity to osmotic stress, cell rigidity (inadequate deformability), deficiency of spectrin or ankyrin, and the severity of the anemia, are statistically highly correlated. 11 refs., 3 tabs.

• Research Organization: Jackson Lab., Bar Harbor, ME (USA)
• DOE Contract Number: AC02-76EV03264
• OSTI ID: 6586627
• Report Number(s): DOE/EV/03264-T3; ON: DE87010458
• Resource Relation: Other Information: Portions of this document are illegible in microfiche products
• Country of Publication: United States
• Language: English

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
62 RADIOLOGY AND NUCLEAR MEDICINE
ANEMIAS
BIOLOGICAL MODELS
CELL MEMBRANES
ERYTHROCYTES
HEREDITARY DISEASES
MICE
ANIMALS
BIOLOGICAL MATERIALS
BLOOD
BLOOD CELLS
BODY FLUIDS
CELL CONSTITUENTS
DISEASES
HEMIC DISEASES
MAMMALS
MATERIALS
MEMBRANES
RODENTS
SYMPTOMS
VERTEBRATES
550900* - Pathology
550600 - Medicine