Analysis Tools
mortality/aging
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• all mice die immediately after birth
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cardiovascular system
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• all E16.5 embryos show a hematoma on the top of the head which grows larger over time (E18.5 and P0)
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craniofacial
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• complete loss of the skull vault at E16.5, E18.5 and P0
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skeleton
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• marked increase of chondrocyte apoptosis noted in the proliferative zone at E14.5 and in the hypertrophic zone at E16.5, as detected by TUNEL staining of humeral growth plates
• upregulation of cleaved (active) caspase-3 in E14.5 chondrocytes, consistent with increased cell apoptosis
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• significant reduction in chondrocyte proliferation rate, as shown by BrdU staining of E14.5 humeral growth plates
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• all E14.5-P0 mice exhibit multiple striking skeletal defects
• however, overall body size is not markedly different from that of controls
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• complete loss of the skull vault at E16.5, E18.5 and P0
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• significant reduction in clavicle length at E18.5
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• severe hypoplasia of the clavicle at E18.5
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• chondrocytes fail to form regular columns in the proliferative zone of humeral growth plates, unlike in controls
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• absence of an organized hypertrophic zone in humeral growth plates at E14.5
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• large spherical chondrocytes are scattered throughout the humeral growth plate, instead of forming the highly organized hypertrophic zone seen in controls
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• significant reduction in the length of all long bones (femur, tibia, humerus, radius and ulna) and their respective bony portions at E18.5
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• blocked (forelimb) or delayed (hindlimb) elongation of distal phalanges at E18.5
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• at E18.5
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short radius
(
J:224370
)
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• at E18.5
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short ulna
(
J:224370
)
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• at E18.5
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short femur
(
J:224370
)
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• at E18.5
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short tibia
(
J:224370
)
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• at E18.5
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• shortened and broadened scapula at E18.5
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• at E18.5
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• shortened, broadened and fused sterna at E18.5
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• fused sterna at E18.5
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• at E18.5
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wide sternum
(
J:224370
)
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• at E18.5
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• chondrocytes are large and spherical instead of discoid, suggesting increased chondrocyte hypertrophy
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• severe reduction of chondrocyte density in humeral growth plates starting at E14.5 and worsening over time
• significant reduction of chondrocyte density in tibial growth plates at E16.5 and E18.5
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• chondrodysplasia
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• severe delay in primary ossification center (POC) formation in the humerus, with no POC noted at E16.5, a partially formed POC at E18.5, and a significantly shorter POC at PO relative to controls
• similar delay in formation of ossification centers in digits (phalange bones)
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• severe defects in intramembranous ossification, as shown by the complete loss of the skull vault and severe clavicle hypoplasia
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limbs/digits/tail
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• blocked (forelimb) or delayed (hindlimb) elongation of distal phalanges at E18.5
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• impaired elongation of distal digits
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• at E18.5
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short radius
(
J:224370
)
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• at E18.5
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short ulna
(
J:224370
)
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• at E18.5
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short femur
(
J:224370
)
|
• at E18.5
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short tibia
(
J:224370
)
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• at E18.5
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• shortened and broadened limbs at E18.5
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short limbs
(
J:224370
)
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• severe forelimb and hindlimb shortening at E18.5
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cellular
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• marked increase of chondrocyte apoptosis noted in the proliferative zone at E14.5 and in the hypertrophic zone at E16.5, as detected by TUNEL staining of humeral growth plates
• upregulation of cleaved (active) caspase-3 in E14.5 chondrocytes, consistent with increased cell apoptosis
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• significant reduction in chondrocyte proliferation rate, as shown by BrdU staining of E14.5 humeral growth plates
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