scholarly journals A Transgenic Mouse Model for Human Sickle Cell Disease

1990 ◽  
Vol 6 ◽  
pp. 6-12
Author(s):  
Richard R. BEHRINGER ◽  
Thomas M. RYAN ◽  
Michael P. REILLY ◽  
Toshio ASAKURA ◽  
Richard D. PALMITER ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Sickle Cell ◽  
Transgenic Mouse Model ◽  
Cell Disease

Mechanisms of vascular instability in a transgenic mouse model of sickle cell disease

2000 ◽  
Vol 279 (6) ◽  
pp. R1949-R1955 ◽  
Author(s):  
K. A. Nath ◽  
V. Shah ◽  
J. J. Haggard ◽  
A. J. Croatt ◽  
L. A. Smith ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Blood Pressure ◽  
Lipid Peroxidation ◽  
Sickle Cell Disease ◽  
Mouse Model ◽  
Systolic Blood Pressure ◽  
Transgenic Mouse ◽  
Sickle Cell ◽  
Transgenic Mouse Model ◽  
Cell Disease

We investigated a transgenic mouse model of sickle cell disease, homozygous for deletion of mouse β-globin and containing transgenes for human βSand βS-antillesglobins linked to the transgene for human α-globin. In these mice, basal cGMP production in aortic rings is increased, whereas relaxation to an endothelium-dependent vasodilator, A-23187, is impaired. In contrast, aortic expression of endothelial nitric oxide synthase (NOS) is unaltered in sickle mice, whereas expression of inducible NOS is not detected in either group; plasma nitrate/nitrite concentrations and NOS activity are similar in both groups. Increased cGMP may reflect the stimulatory effect of peroxides (an activator of guanylate cyclase), because lipid peroxidation is increased in aortae and in plasma in sickle mice. Despite increased vascular cGMP levels in sickle mice, conscious systolic blood pressure is comparable to that of aged-matched controls; sickle mice, however, evince a greater rise in systolic blood pressure in response to nitro-l-arginine methyl ester, an inhibitor of NOS. Systemic concentrations of the vasoconstrictive oxidative product 8-isoprostane are increased in sickle mice. We conclude that vascular responses are altered in this transgenic sickle mouse and are accompanied by increased lipid peroxidation and production of cGMP; we suggest that oxidant-inducible vasoconstrictor systems such as isoprostanes may oppose nitric oxide-dependent and nitric oxide-independent mechanisms of vasodilatation in this transgenic sickle mouse. Destabilization of the vasoactive balance in the sickle vasculature by clinically relevant states may predispose to vasoocclusive disease.

scholarly journals Activation of blood platelets in a transgenic mouse model of sickle cell disease

2007 ◽  
Vol 21 (6) ◽  
Author(s):  
Renata K Polanowska‐Grabowska ◽  
Robert A. Figler ◽  
Melissa M Marshall ◽  
Kori Wallace ◽  
Adrian R. L. Gear ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Sickle Cell ◽  
Blood Platelets ◽  
Transgenic Mouse Model ◽  
Cell Disease

scholarly journals Dietary  -3 fatty acids protect against vasculopathy in a transgenic mouse model of sickle cell disease

Haematologica ◽  
2015 ◽  
Vol 100 (7) ◽  
pp. 870-880 ◽  
Author(s):  
B. T. Kalish ◽  
A. Matte ◽  
I. Andolfo ◽  
A. Iolascon ◽  
O. Weinberg ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Sickle Cell Disease ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Sickle Cell ◽  
Transgenic Mouse Model ◽  
Cell Disease

scholarly journals Towards a transgenic mouse model of sickle cell disease: hemoglobin SAD.

1991 ◽  
Vol 10 (11) ◽  
pp. 3157-3165 ◽  
Author(s):  
M. Trudel ◽  
N. Saadane ◽  
M.C. Garel ◽  
J. Bardakdjian-Michau ◽  
Y. Blouquit ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Sickle Cell ◽  
Transgenic Mouse Model ◽  
Cell Disease

scholarly journals Magnetic resonance evidence of hypoxia in a homozygous alpha-knockout of a transgenic mouse model for sickle cell disease.

1996 ◽  
Vol 98 (11) ◽  
pp. 2450-2455 ◽  
Author(s):  
M E Fabry ◽  
R P Kennan ◽  
C Paszty ◽  
F Costantini ◽  
E M Rubin ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Sickle Cell ◽  
Transgenic Mouse Model ◽  
Cell Disease

scholarly journals A transgenic mouse model of sickle cell disorder

Nature ◽  
1990 ◽  
Vol 343 (6254) ◽  
pp. 183-185 ◽  
Author(s):  
David R. Greaves ◽  
Peter Fraser ◽  
Miguel A. Vidal ◽  
Michael J. Hedges ◽  
David Ropers ◽  
...  
Keyword(s):  
Mouse Model ◽  
Transgenic Mouse ◽  
Sickle Cell ◽  
Transgenic Mouse Model ◽  
Cell Disorder
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