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Musculoskeletal, Absent mm.-typ. unilateral typ. sternocostal pec. maj head palm. longus, trapezius-common Prune Belly Syndrome-no ab mm. assoc. w/ urinary bladder defects Congenital Torticollis (wryneck)- fixed rotated/tilted head-birth trauma ???? Regulation 1.MyoD family specific x-factors 2.signaling molecules, i.e. FGF keeps myoblasts dividing w/draw FGF-fusion into myotubes Pattern determined by CT around myo., Derived from paraxial mesoderm (somites)- dermomyotome-dorsolateral somite ventrolateral lip-limb (hypaxial) mm. limb and body wall dorsomedial lip-back (epaxial) mm. myoblasts derived from each lip migrate to final dest. form pre-muscle masses subdivided into muscles tendons from sclerotome ???? Muscle Innervation Head & Neck-cranial nerves Trunk & Limbs-spinal nerves innervated early as mm. masses, Mesen. cells b/w these halves->IV disc notochord of this region->nuc. pulposus ???? Spina Bifida-vertebral arches don't fuse neural tissue exposed Achondroplasia-FGF-receptor 3 gene mutation early maturation of epiphys. plate, dwarfism mental development normal Marfan's disease-alters fibrillin production (small fibrils around elastic fibers), Molec. Regulation to dermomyotome (DM) & sclerotome (of somite) ???? Wnt proteins- from dorsal neur. tube induce Myf5 (mm. x-factor) express in dorsomed. DM epaxial mm., Derived from paraxial mesoderm (somites)- dermomyotome-dorsolateral somite ventrolateral lip-limb (hypaxial) mm. limb and body wall dorsomedial lip-back (epaxial) mm. myoblasts derived from each lip migrate to final dest. form pre-muscle masses subdivided into muscles tendons from sclerotome ???? BMP4, FGFs from lat. plate meso to ventrolat. DM->MyoD hypaxial mm, Limb Field - 4th week Somatic lat. plate mesoderm produces FGF-10 Induce ectoderm to grow into limb bud mesenchyme core-mesoderm deriv distal end of bud Apical Ectodermal Ridge (AER) inhibits diff. of mesenchyme promotes limb bud growth mesen. far from AER diff. cart. core surr. by mm. masses secretes FGFs 2,4,8 to lengthen limb amelia (no limb), meromelia (short limb) result from no FGFs from ectoderm, Apical Ectodermal Ridge (AER) inhibits diff. of mesenchyme promotes limb bud growth mesen. far from AER diff. cart. core surr. by mm. masses secretes FGFs 2,4,8 to lengthen limb amelia (no limb), meromelia (short limb) result from no FGFs from ectoderm Posterior surface of limb bud Zone of Polarizing Activity (ZPA) post. surface ectoderm of limb bud polarizes A-P axis and digits via Shh and retinoic acid signals called ZPA morphogens induce Homeobox genes for digit location diplopodia- mirror-image duplication of digits, Derived from splanchnic meso. gut and gut derivs sm. m for dorsal aorta, large aa. & neural crest sm. m for dorsal aorta, large aa. sm. m for coronary aa. ???? Cardiac mm. splanchnic meso near heart tube myoblasts att. via IC disc precursors dev. like sk. m. but don't fuse Purkinje fibers deriv. from mm., Muscular System development Smooth/Cardiac M. Derived from splanchnic meso. gut and gut derivs sm. m for dorsal aorta, large aa. & neural crest sm. m for dorsal aorta, large aa. sm. m for coronary aa., Molec. Regulation to dermomyotome (DM) & sclerotome (of somite) ???? Neurotrophin-4 (NT-4) from dorsal neur. tube to dorsal DM->dermis