Phenotypes Associated with This Genotype

Genotype
MGI:3716189

cx16

• Allelic Composition: Dmd^mdx/?; Fgf2^tm1Zllr/Fgf2^tm1Zllr; Fgf6^tm1Thbr/Fgf6^tm1Thbr
• Genetic Background: involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * C57BL/6 * C57BL/10ScSn

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

muscle

muscle phenotype ( J:85088 )

N • growth and differentiation kinetics of myogenic cells are comparable to wild-type

abnormal skeletal muscle morphology ( J:85088 )

• pale areas in muscle contain extensive connective tissue

abnormal skeletal muscle fiber morphology ( J:85088 )

• when mice are injected with the diazo dye EBD, dye is taken up by damaged muscle fibers to varying degrees, but preferentially into the hindlimb; uptake occurs because of focal breakdown of plasmalemma which is an early event in necrosis

• some muscles are stained entirely indicating massive damage to the muscle fibers of the diaphragm and gluteus maximus whereas muscles in wild-type mice do not take up dye

increased variability of skeletal muscle fiber size ( J:85088 )

• in pale areas of muscles, remaining myotubes show large variation in caliber size

skeletal muscle fiber necrosis ( J:85088 )

• numerous necrotic fibers are present

decreased satellite cell number ( J:85088 )

• myotubes have slightly reduced percentage of satellite cells with respect to myotube nuclei in wild-type (3.05% vs 3.56%)

dystrophic muscle ( J:85088 )

• severe abnormalities are observed

abnormal muscle physiology ( J:85088 )

• mutants display palpable stiffness of the musculature, most evident in pelvic and shoulder girdle, at up to 6 months of age

skeleton

abnormal spine curvature ( J:85088 )

• up to 6 months of age, mice do not show clinical signs of severe dystrophy except for dorsal-ventral curvature of the spine