mortality/aging
homeostasis/metabolism
cardiovascular system
• dramatic overgrowth
|
growth/size/body
• dramatic overgrowth
|
• kidneys that do form are significantly enlarged
|
renal/urinary system
• kidneys may display agenesis or over growth, with both conditions often seen in the same animal
• unilateral or bilateral renal agenesis is seen in 64% of mice
• kidneys that do form have an expanded population of mesenchymal progenitor cells
|
• kidneys that do form are significantly enlarged
|
• the Wolffian duct remains detached from the ureteric bud at E13.5 in some mice
• absence of ureteric bud derived structures at E13.5 in some mice
|
• expression analysis indicates that the metanephric mesenchyme is specified but fails to condense and undergo epithelialization
|
• widespread apoptosis is seen in many mice
|
• absence of ureteric bud derived structures at E13.5 in some mice
|
embryo
• widespread apoptosis is seen in many mice
|
• the Wolffian duct remains detached from the ureteric bud at E13.5 in some mice
|
skeleton
• dramatic bone overgrowth
|
• malformation of the postnatal suture
|
• abnormally distant ossification fronts in the perinatal cranial vault
|
• enlarged cranial vault
|
• discontinuous cortical bone layer at E15.5
|
• malformed or absent
|
bowed radius
(
J:173242
)
|
• enlarged radius
|
• enlarged and bowed
|
bowed ulna
(
J:173242
)
|
• massive increases in mineralized matrix
|
• decreased mineralization of the trabecular area in long bones perinatally
• massive increases in mineralized matrix in the compact bone
|
• deficiency in endochondral bone ossification
|
• asymmetrical distribution of ossification centers
|
• delayed ossification of the long bones at E14.5
• discontinuous cortical bone layer in long bones at E15.5
|
• deficiency in intramembranous bone ossification
|
• abnormally distant ossification fronts in the perinatal cranial vault
|
adipose tissue
• severely reduced
|
• severely reduced
|
• hypoplastic adipose tissue
|
hematopoietic system
immune system
craniofacial
• malformation of the postnatal suture
|
• abnormally distant ossification fronts in the perinatal cranial vault
|
• enlarged cranial vault
|
limbs/digits/tail
• malformed or absent
|
bowed radius
(
J:173242
)
|
• enlarged radius
|
• enlarged and bowed
|
bowed ulna
(
J:173242
)
|
cellular
• widespread apoptosis is seen in many mice
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
osteopathia striata with cranial sclerosis | DOID:0060886 |
OMIM:300373 |
J:173242 |