mortality/aging
• mice with abnormal heart looping die between E9.5 and E11.5
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cardiovascular system
• increased cell proliferation is seen in various regions of the heart at E14.5, E15.5, E17.5 and P0
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• about 15% of mice show looping abnormalities including straight heart tubes, incomplete looping, and variable dilations along the heart tube
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• enlarged coronary vessels in newborns
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• a thickened interventricular septum is seen in newborns
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• seen in newborns
• increase in thickness is due to hyperplasia not hypertrophy as myocyte size is similar to controls
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muscle
N |
• despite being derived from Lbx1+ precursor cells diaphragm and tongue muscle morphology appears normal
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• migration of most lacZ expressing precursor cells is disrupted with lacZ+ cells
• ectopically distributed along the rostral caudal axis in the intermediate and lateral plate mesoderm at E10.5
• migration of precursor cells to some ventral limb muscles, tongue and diaphragm appears slightly delayed
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• severe limb muscle defects
• hindlimbs contain only a few scattered myocytes
• no myotubes are detected in the dorsal part of the forelimbs, while the ventral flexor muscles are mostly present but a few are absent or reduced in size
• affected forelimb ventral muscles (m. flexor digitorum superficalis et profundi, m. opponens pollicis and m. adductor pollicic) are at the distal end of the limb
• most shoulder muscles are also missing
• remaining forelimb muscles contain fewer myofibers with enlarged myotubes containing centrally located nuclei
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limbs/digits/tail
• most ventral flexor muscles are present but a few are absent or reduced in size
• abnormally bent morphology in newborns
• no myotubes are detected in the dorsal part of the forelimbs
• affected forelimb ventral muscles (m. flexor digitorum superficalis et profundi, m. opponens pollicis and m. adductor pollicic) are at the distal end of the limb
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• thin compared to heterozygous littermates
• hindlimbs contain only a few scattered myocytes
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• severe limb muscle defects
• hindlimbs contain only a few scattered myocytes
• no myotubes are detected in the dorsal part of the forelimbs, while the ventral flexor muscles are mostly present but a few are absent or reduced in size
• affected forelimb ventral muscles (m. flexor digitorum superficalis et profundi, m. opponens pollicis and m. adductor pollicic) are at the distal end of the limb
• most shoulder muscles are also missing
• remaining forelimb muscles contain fewer myofibers with enlarged myotubes containing centrally located nuclei
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embryo
• seen in mice with abnormal heart looping
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behavior/neurological
• no movement of the forelimbs is seen although spontaneous trunk movements are present
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• no movement of the hindlimbs is seen although spontaneous trunk movements are present
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cellular
• migration of most lacZ expressing precursor cells is disrupted with lacZ+ cells
• ectopically distributed along the rostral caudal axis in the intermediate and lateral plate mesoderm at E10.5
• migration of precursor cells to some ventral limb muscles, tongue and diaphragm appears slightly delayed
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