mortality/aging
|
• death by 3 weeks of age
|
hematopoietic system
|
• in thymus
|
|
• in both thymus and spleen
|
|
• with decreased numbers of red blood cells and few megakaryocytes observed
|
small spleen
(
J:73418
)
|
• lymphoid counts substantially reduced
|
|
• with decreased cortical-medullary demarcation
|
small thymus
(
J:73418
)
immune system
|
• in thymus
|
|
• in both thymus and spleen
|
|
• with decreased numbers of red blood cells and few megakaryocytes observed
|
small spleen
(
J:73418
)
|
• lymphoid counts substantially reduced
|
|
• with decreased cortical-medullary demarcation
|
small thymus
(
J:73418
)
|
• evidence of inflammatory cells, thickening and shedding of airway epithelium and occlusion of airways with mucous and cellular debris
|
renal/urinary system
|
• increased red blood cells within glomeruli
|
|
• increased red blood cells within convoluted tubules
|
respiratory system
|
• evidence of inflammatory cells, thickening and shedding of airway epithelium and occlusion of airways with mucous and cellular debris
|
|
• evident beginning at postnatal day 12 and progressively labored breathing until death
|
skeleton
|
• enlarged costochondral junctions
• severe rib curvature
|
cellular
|
• in thymus
|
|
• in both thymus and spleen
|
Mouse Models of Human Disease |
OMIM ID | Ref(s) | |
| Severe Combined Immunodeficiency, Autosomal Recessive, T Cell-Negative, B Cell-Negative, Nk Cell-Negative, Due to Adenosine Deaminase Deficiency | 102700 | J:73418 | |