All Phenotypes Fgfr3<tm4.1Cxd> MGI Mouse

mortality/aging ( J:70061 )

N	• Background Sensitivity: compared to offspring of heterozygotes backcrossed to Black Swiss, crosses of male mutants to female CD-1 mice result in <10% survival to 3 months of severely affected mutants

neonatal lethality, complete penetrance ( J:63198 )

• die within a few hours to 1 day after birth

increased chondrocyte proliferation ( J:63198 )

• chondrocyte proliferation in growth plates is twice that of wild-type at E15.5, however little difference is seen at E18.5

abnormal osteoblast physiology ( J:63198 )

• exhibit increased osteoblast activity at E15.5 and E18.5

abnormal long bone morphology ( J:63198 )

• long bone abnormalities are seen as early as E14, with increased long bone diameter at E14-15 and shorter bones later

abnormal ulna morphology ( J:63198 )

bowed ulna ( J:63198 )

• exhibit marked bowing of the ulna

abnormal long bone diaphysis morphology ( J:63198 )

• ossified region of the diaphysis is shortened at P1

abnormal long bone epiphyseal plate morphology ( J:63198 )

• differentiation of growth plate chondrocytes is suppressed starting at early embryonic stages

• resting chondrocyte-like cells with small and round appearance are randomly located in the proliferation and hypertrophic zones

• the medial portion of growth plates curve toward the diaphysis into primary spongiosa at E15.5 and P1

• exhibit thick and extended perichondrium in P1 growth plates

• 15-20% increase in the number of cells in growth plates at E14-15

abnormal long bone epiphyseal plate proliferative zone ( J:63198 )

• very short or no stacked-cell columnar organization of proliferating chondrocytes at P1

• chondrocytes in the resting and proliferating zones are smaller and have a tightly packed appearance at E14-15

abnormal long bone hypertrophic chondrocyte zone ( J:63198 )

• hypertrophic chondrocytes show incomplete maturation without full enlargement at E14-15

decreased length of long bones ( J:63198 )

short humerus ( J:63198 )

• length of humerus is 77% of wild-type

short ulna ( J:63198 )

• length of ulna is 80% of wild-type

short femur ( J:63198 )

• length of femur is 88% of wild-type

short tibia ( J:63198 )

• length of tibia is 77% of wild-type

• exhibit marked bowing of the tibia

increased diameter of long bones ( J:63198 )

wide sternum ( J:63198 )

• widening of the sterni

abnormal costal cartilage morphology ( J:63198 )

• costal cartilage is widened and shows chondrocytes that are tightly packed

• proliferation in costal cartilage is increased 258% compared to wild-type at E18.5

abnormal thoracic cage shape ( J:63198 )

• caudal widening

small thoracic cage ( J:63198 )

• small rib cage that shows caudal widening

abnormal spine curvature ( J:63198 )

• exhibit curvature of the axial skeleton

abnormal chondrocyte differentiation ( J:63198 )

• chondrocyte differentiation from the resting to proliferating, and to hypertrophic states, is suppressed

abnormal bone ossification ( J:63198 )

• exhibit decreased ossification in the spine, ribs and epiphyses of the long bones

failure of sternum ossification ( J:63198 )

• no ossification of sternebrae seen at P1

impaired lung alveolus development ( J:63198 )

• lungs at E18.5 or earlier are normal, however after birth, the alveoli are not completely formed