EurekaMag.com logo
+ Translate

Spectrin deficient inherited hemolytic anemias in the mouse characterization by spectrin synthesis and messenger rna activity in reticulocytes


, : Spectrin deficient inherited hemolytic anemias in the mouse characterization by spectrin synthesis and messenger rna activity in reticulocytes. Cell 37(3): 721-730

Spectrin synthesis and mRNA activity were investigated in mice homozygous and heterozygous for 6 mutations occurring at 3 distinct loci (nb, ja, sph). When homozygous, these mutations cause severe hemolytic anemias that are characterized by specific spectrin deficiencies. Evidently, the primary effect of the nb mutation is a deficiency of another erythrocyte membrane skeletal protein, ankyrin. The severe deficiency of spectrin in the red blood cells of ja/ja mice is the result of a .beta. spectrin defect. Analysis of spectrin synthesis in mice homozygous and heterozygous for several alleles of sph indicates that the sph locus is the structural gene locus for .alpha. spectrin. The sph locus was mapped to mouse chromosome 1.

(PDF 0-2 workdays service)

Accession: 006459998

Submit PDF Full Text: Here


Submit PDF Full Text

No spam - Every submission is manually reviewed

Due to poor quality, we do not accept files from Researchgate

Submitted PDF Full Texts will always be free for everyone
(We only charge for PDFs that we need to acquire)

Select a PDF file:
Close
Close

Related references

Bodine, D.M.; Birkenmeier, C.S.; Barker, J.E., 1984: Spectrin deficient inherited hemolytic anemias in the mouse: characterization by spectrin synthesis and mRNA activity in reticulocytes. We have investigated spectrin synthesis and mRNA activity in mice homozygous and heterozygous for six mutations occurring at three distinct loci (nb, ja, sph). When homozygous, these mutations cause severe hemolytic anemias that are characterized...

Wichterle, H.; Palek, J.; Hanspal, M.; Smockova, Y.; Brabec, V.; Jarolim, P., 1995: Two recessively inherited defects of alpha spectrin underlie a severe, spectrin-deficient spherocytic hemolytic anemia. Blood 86(10 SUPPL 1): 468A

Birkenmeier, C.S.; McFarland-Starr, E.C.; Barker, J.E., 1988: Chromosomal location of three spectrin genes: relationship to the inherited hemolytic anemias of mouse and man. Three genetic loci in the mouse affect the synthesis and assembly of the erythrocyte membrane skeleton. The spherocytosis and jaundiced loci affect the membrane skeletal protein known as spectrin. The normoblastosis locus affects the spectrin bind...

Agre P.; Bennett V., 1983: Spectrin deficient spherocytosis is spectrin synthesis reduced or is the spectrin unstable?. Journal of Cellular Biochemistry Supplement (7 PART B): 50

Lux, S.E.; Pease, B.; Tomaselli, M.B.; John, K.M.; Bernstein, S.E., 1979: Hemolytic anemias associated with deficient or dysfunctional spectrin. Elliptocytes from patients with hereditary elliptocytosis (HE) form elliptical ghosts and membrane skeletons. The composition of HE skeletons is quantitatively normal; however, in some but not all kindreds the major membrane skeletal protein, spec...

Anonymous, 1982: Spectrin and red cell shape 1 neuraminidase reverses exo vesiculation in genetically spectrin deficient mouse red blood cells

Hassoun, H.; Vassiliadis, J.N.; Murray, J.; Jarolim, P.; Brabec, V.; Palek, J., 1994: Beta Spectrin Tabor A nonsense mutation within the beta spectrin gene associated with dominantly inherited spherocytosis and spectrin deficiency. Blood 84(10 SUPPL 1): 6A

Wurzinger L.J.; Schmid Schoenbein H.; Grebe R.; Wolf M.; Wolff H.; Heller K., 1986: Spectrin glycocalyx antagonism effects of neuraminidase treatment on curvature of membrane ghosts of genetically spectrin deficient mouse red blood cells. Biorheology 23(3): 257

Anonymous, 2007: Identification and characterization of alpha-spectrin mutations associated with inherited hemolytic anemia.

Winkelmann, J.C.; Costa, F.F.; Linzie, B.L.; Forget, B.G., 1990: Beta spectrin in human skeletal muscle tissue specific differential processing of 3' beta spectrin pre messenger rna generates a beta spectrin isoform with a unique carboxyl terminus. Spectrin, an important component of the mammalian erythrocyte membrane skeleton, is a heterodimeric protein with .alpha. and .beta. subunits of 280 and 246 kDa, respectively. Spectrin-like proteins have also been demonstrated in a wide variety of...