RGD Reference Report - Hematopoietic cells from -spectrin-deficient mice are sufficient to induce thrombotic events in hematopoietically ablated recipients. - Rat Genome Database

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Hematopoietic cells from -spectrin-deficient mice are sufficient to induce thrombotic events in hematopoietically ablated recipients.

Authors: Wandersee, NJ  Lee, JC  Kaysser, TM  Bronson, RT  Barker, JE 
Citation: Wandersee NJ, etal., Blood. 1998 Dec 15;92(12):4856-63.
RGD ID: 11059524
Pubmed: PMID:9845553   (View Abstract at PubMed)

Thrombotic events are life-threatening complications of human hemolytic anemias such as paroxysmal nocturnal hemoglobinuria, sickle cell disease, and thalassemia. It is not clear whether these events are solely influenced by aberrant hematopoietic cells or also involve aberrant nonhematopoietic cells. Spherocytosis mutant (Spna1(sph)/Spna1(sph); for simplicity referred to as sph/sph) mice develop a severe hemolytic anemia postnatally due to deficiencies in -spectrin in erythroid and other as yet incompletely defined nonerythroid tissues. Thrombotic lesions occur in all adult sph/sph mice, thus providing a hematopoietically stressed model in which to assess putative causes of thrombus formation. To determine whether hematopoietic cells from sph/sph mice are sufficient to initiate thrombi, bone marrow from sph/sph or +/+ mice was transplanted into mice with no hemolytic anemia. One set of recipients was lethally irradiated; the other set was genetically stem cell deficient. All mice implanted with sph/sph marrow, but not +/+ marrow, developed severe anemia and histopathology typical of sph/sph mice. Histological analyses of marrow recipients showed that thrombi were present in the recipients of sph/sph marrow, but not +/+ marrow. The results indicate that the -spectrin-deficient hematopoietic cells of sph/sph mice are the primary causative agents of the thrombotic events.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
SPTA1Humanthrombosis  ISOSpta1 (Mus musculus) RGD 
Spta1Ratthrombosis  ISOSpta1 (Mus musculus) RGD 
Spta1Mousethrombosis  IMP  RGD 

Objects Annotated

Genes (Rattus norvegicus)
Spta1  (spectrin, alpha, erythrocytic 1)

Genes (Mus musculus)
Spta1  (spectrin alpha, erythrocytic 1)

Genes (Homo sapiens)
SPTA1  (spectrin alpha, erythrocytic 1)


Additional Information