respiratory system
• two-fold reduction in the size of the caudal digastric muscle
|
skeleton
• lower teeth are longer and more rounded than in wild-type mice
|
mortality/aging
• 3 of 35 mice died at birth
|
• 7 of 29 mice died between 15 and 24 days after birth
|
nervous system
N |
• rhombomere structure, neural crest cell production and neural crest migration are normal
|
• neurons that are present along the medial margin of r4 and r5 are either absent or reduced in number with anteriorly directed axons fibers not detected and dispersed neuronal cell bodies and fibers of r4 exiting the hindbrain without forming tightly bound fasciculation as observed in wild-type mice
• the caudal and ventrolateral migration of medial neurons around the parasympathetic neurons in r5 is absent
|
• 3 of five mice have undetectable facial motor nuclei
|
• 2 of five mice have small facial motor nuclei compared to in wild-type mice
|
• mice exhibit variable defects in branching of the CN-VII including a 2- to 3-fold reduction in nerve branch diameter and missing branches including the orbicularis oris, the mandibular branch and buccal branch
• however, despite defects in the motor component of the facial nerve, the sensory component of the facial nerve is normal
• the nerve that innervates the stapes and stapedius muscle is reduced in size and dissociated from the muscle
|
craniofacial
• lower teeth are longer and more rounded than in wild-type mice
|
narrow face
(
J:36235
)
• some mice exhibit a narrow face
|
• two-fold reduction in the size of the buccinator muscle
|
• two-fold reduction in the size of the depressor anguli oris muscle
|
• mice exhibit abnormal mass and shape of the masseter muscles
|
• mice exhibit abnormal mass and shape of the temporalis muscle
|
• two-fold reduction in the size of the zygomatic muscles
|
• absence of the nasolabialis and labii maxillaries muscle
|
• mice exhibit degeneration of facial muscles including a two-fold reduction in the size of the zygomatic, buccinator, depressor anguli oris and caudal digastric muscles and an absence of the nasolabialis and labii maxillaris muscle
|
• some mice exhibit a receded lower lip
|
behavior/neurological
• mice exhibit facial paralysis including the inability to move whiskers or nose, to close eye lids in response to touch or to move ears in response to sound or touch
|
muscle
• two-fold reduction in the size of the buccinator muscle
|
• two-fold reduction in the size of the depressor anguli oris muscle
|
• mice exhibit abnormal mass and shape of the masseter muscles
|
• mice exhibit abnormal mass and shape of the temporalis muscle
|
• two-fold reduction in the size of the zygomatic muscles
|
• absence of the nasolabialis and labii maxillaries muscle
|
• mice exhibit degeneration of facial muscles including a two-fold reduction in the size of the zygomatic, buccinator, depressor anguli oris and caudal digastric muscles and an absence of the nasolabialis and labii maxillaris muscle
|
• two-fold reduction in the size of the caudal digastric muscle
|
growth/size/body
• lower teeth are longer and more rounded than in wild-type mice
|
narrow face
(
J:36235
)
• some mice exhibit a narrow face
|
• two-fold reduction in the size of the buccinator muscle
|
• two-fold reduction in the size of the depressor anguli oris muscle
|
• mice exhibit abnormal mass and shape of the masseter muscles
|
• mice exhibit abnormal mass and shape of the temporalis muscle
|
• two-fold reduction in the size of the zygomatic muscles
|
• absence of the nasolabialis and labii maxillaries muscle
|
• mice exhibit degeneration of facial muscles including a two-fold reduction in the size of the zygomatic, buccinator, depressor anguli oris and caudal digastric muscles and an absence of the nasolabialis and labii maxillaris muscle
|
• some mice exhibit a receded lower lip
|
• some mice exhibit runting
|
cellular
• neurons that are present along the medial margin of r4 and r5 are either absent or reduced in number with anteriorly directed axons fibers not detected and dispersed neuronal cell bodies and fibers of r4 exiting the hindbrain without forming tightly bound fasciculation as observed in wild-type mice
• the caudal and ventrolateral migration of medial neurons around the parasympathetic neurons in r5 is absent
|