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Phenotypes Associated with This Genotype
Genotype
MGI:3805200
Allelic
Composition
Hrastm2Xbr/Hrastm2Xbr
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hrastm2Xbr mutation (1 available); any Hras mutation (21 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Virgin Hrastm2Xbr/Hrastm2Xbr females display mammary gland hyperplasia

tumorigenesis
N
• most (19/20) mice are tumor free at 18 months of age whereas about 10% pf humans with a comparable mutation develop various tumors (J:137729)
• most (19/20) mice are tumor free at 18 months of age whereas about 10% pf humans with a comparable mutation develop various tumors (J:137729)
• one mouse in 20 has a mammary adenocarcinoma at 18 months (J:137729)
• epithelial in origin (J:137729)
• one mouse in 20 has a mammary adenocarcinoma at 18 months (J:137729)
• epithelial in origin (J:137729)

endocrine/exocrine glands
• increased alveolar proliferation (J:137729)
• slight decrease in adipose tissue (J:137729)
• ductal ectasia (J:137729)
• mild periductal-alveolar fibrosis (J:137729)
• increased alveolar proliferation (J:137729)
• slight decrease in adipose tissue (J:137729)
• ductal ectasia (J:137729)
• mild periductal-alveolar fibrosis (J:137729)
• increased ductal branching (J:137729)
• greater ductal-lobulillar development (J:137729)
• increased ductal branching (J:137729)
• greater ductal-lobulillar development (J:137729)
• in females at around 4 months of age (J:137729)
• in females at around 4 months of age (J:137729)

craniofacial
• depression of anterior frontal bones (J:137729)
• depression of anterior frontal bones (J:137729)
• shortened maxilla (J:137729)
• shortened molar process (J:137729)
• shortened maxilla (J:137729)
• shortened molar process (J:137729)
• facial dysmorphia (J:137729)
• defects observed neonatally (J:137729)
• facial dysmorphia (J:137729)
• defects observed neonatally (J:137729)
• engrossed lips due to accumulation of adipocytes between skeletal muscle fascicules (J:137729)
• more sebaceous glands (J:137729)
• shorter elastin fibers (J:137729)
• engrossed lips due to accumulation of adipocytes between skeletal muscle fascicules (J:137729)
• more sebaceous glands (J:137729)
• shorter elastin fibers (J:137729)
• shortened and depressed nasal bridge (J:137729)
• narrowed choanae (J:137729)
• shortened and depressed nasal bridge (J:137729)
• narrowed choanae (J:137729)

cardiovascular system
N
• heart rate normal (J:137729)
• heart rate normal (J:137729)
• blood vessels generally more reactive to angiotensin II (J:137729)
• blood vessels generally more reactive to angiotensin II (J:137729)
• develop severe thickening and remodeling of aorta media over time (J:137729)
• develop severe thickening and remodeling of aorta media over time (J:137729)
• changes less pronounced at 6 months than at 4 months (J:137729)
• sarcomere organization normal (J:137729)
• changes less pronounced at 6 months than at 4 months (J:137729)
• sarcomere organization normal (J:137729)
• enlarged heart chambers at 4 months (J:137729)
• overall increase in size of walls and chambers (J:137729)
• cell size and density normal (J:137729)
• enlarged heart chambers at 4 months (J:137729)
• overall increase in size of walls and chambers (J:137729)
• cell size and density normal (J:137729)
• enlarged (J:137729)
• other heart valves normal (J:137729)
• enlarged (J:137729)
• other heart valves normal (J:137729)
• enlarged heart chambers at 4 months (J:137729)
• enlarged heart chambers at 4 months (J:137729)
• increased cardiomyocyte size (J:137729)
• elevated interstitial collagen deposition (J:137729)
• increased cardiomyocyte size (J:137729)
• elevated interstitial collagen deposition (J:137729)
• overall increase in size of walls and chamber (J:137729)
• cell size and density normal (J:137729)
• overall increase in size of walls and chamber (J:137729)
• cell size and density normal (J:137729)
• systemic hypertension (J:137729)
• at least partially due to elevated circulating levels of angiotensin II (J:137729)
• pulmonary blood pressure is normal (J:137729)
• systemic hypertension (J:137729)
• at least partially due to elevated circulating levels of angiotensin II (J:137729)
• pulmonary blood pressure is normal (J:137729)
• anesthetized and conscious (J:137729)
• anesthetized and conscious (J:137729)
• anesthetized and conscious (J:137729)
• anesthetized and conscious (J:137729)

renal/urinary system
N
• urine production normal (J:137729)
• sodium, potassium, chlorine excretion all normal (J:137729)
• urine production normal (J:137729)
• sodium, potassium, chlorine excretion all normal (J:137729)

homeostasis/metabolism
• elevated circulating levels of angiotensin II (J:137729)
• elevated circulating levels of angiotensin II (J:137729)

respiratory system
N
• breathing normal (J:137729)
• breathing normal (J:137729)

skeleton
• depression of anterior frontal bones (J:137729)
• depression of anterior frontal bones (J:137729)
• shortened maxilla (J:137729)
• shortened molar process (J:137729)
• shortened maxilla (J:137729)
• shortened molar process (J:137729)

integument
• increased alveolar proliferation (J:137729)
• slight decrease in adipose tissue (J:137729)
• ductal ectasia (J:137729)
• mild periductal-alveolar fibrosis (J:137729)
• increased alveolar proliferation (J:137729)
• slight decrease in adipose tissue (J:137729)
• ductal ectasia (J:137729)
• mild periductal-alveolar fibrosis (J:137729)
• increased ductal branching (J:137729)
• greater ductal-lobulillar development (J:137729)
• increased ductal branching (J:137729)
• greater ductal-lobulillar development (J:137729)
• in females at around 4 months of age (J:137729)
• in females at around 4 months of age (J:137729)
• distention and rounding of vibrissal follicles (J:137729)
• distention and rounding of vibrissal follicles (J:137729)

growth/size/body
• facial dysmorphia (J:137729)
• defects observed neonatally (J:137729)
• facial dysmorphia (J:137729)
• defects observed neonatally (J:137729)
• engrossed lips due to accumulation of adipocytes between skeletal muscle fascicules (J:137729)
• more sebaceous glands (J:137729)
• shorter elastin fibers (J:137729)
• engrossed lips due to accumulation of adipocytes between skeletal muscle fascicules (J:137729)
• more sebaceous glands (J:137729)
• shorter elastin fibers (J:137729)
• shortened and depressed nasal bridge (J:137729)
• narrowed choanae (J:137729)
• shortened and depressed nasal bridge (J:137729)
• narrowed choanae (J:137729)

Mouse Models of Human Disease
OMIM ID Ref(s)
Costello Syndrome; CSTLO 218040 J:137729


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory