Phenotypes Associated with This Genotype

Genotype
MGI:3789312

• Allelic Composition: Lama2^dy/Lama2^dy
• Genetic Background: B6.129P1-Lama2^dy/J

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

hearing/vestibular/ear

abnormal bony labyrinth ( J:101670 )

• spaces normally filled with perilymph and endolymph fluids are obliterated with a fibrotic debris

abnormal organ of Corti morphology ( J:101670 )

• the structural integrity of the organ of Corti is altered with supporting and hair cells not exhibiting their normal vertical height and a slight compression of the organ, especially at the apical half turn

abnormal cochlear hair cell morphology ( J:101670 )

• hair cells in the organ of Corti not exhibiting their normal vertical height

absent cochlear hair cells ( J:101670 )

abnormal organ of Corti supporting cell morphology ( J:101670 )

• supporting not exhibiting their normal vertical height

cochlear degeneration ( J:101670 )

stria vascularis degeneration ( J:101670 )

abnormal inner ear vestibule morphology ( J:101670 )

• mice exhibit a degeneration of vestibular structures

absent vestibular hair cells ( J:101670 )

increased or absent threshold for auditory brainstem response ( J:101670 )

• mice exhibit an increase in brainstem auditory threshold compared to wild-type mice

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:101670 )

• mice exhibit impaired motor activity

• however, no circling or head twisting is observed

abnormal motor coordination/balance ( J:5000 )

• mice stumble when walking

nervous system

abnormal cochlear hair cell morphology ( J:101670 )

• hair cells in the organ of Corti not exhibiting their normal vertical height

absent cochlear hair cells ( J:101670 )

absent vestibular hair cells ( J:101670 )

abnormal cochlear nerve morphology ( J:101670 )

• few if any nerve fibers remain in the ear

decreased nerve conduction velocity ( J:5948 )

craniofacial

abnormal cranium morphology ( J:129632 )

• the posterior portion of the skull is slightly expanded while the medial portion is narrowed

• mice exhibit wider braincases, a slight flexion in the middle and rotated mandibles

abnormal bony labyrinth ( J:101670 )

• spaces normally filled with perilymph and endolymph fluids are obliterated with a fibrotic debris

increased cranium width ( J:129632 )

• skull width relative to skull length increase is more pronounced than in Lama2^dy-2J homozygotes

reproductive system

decreased testis weight ( J:5000 )

♂

growth/size/body

decreased body weight ( J:5000 )

• drastic

homeostasis/metabolism

increased circulating prolactin level ( J:5000 )

muscle

muscle weakness ( J:101670 )

endocrine/exocrine glands

decreased testis weight ( J:5000 )

♂

skeleton

abnormal cranium morphology ( J:129632 )

• the posterior portion of the skull is slightly expanded while the medial portion is narrowed

• mice exhibit wider braincases, a slight flexion in the middle and rotated mandibles

abnormal bony labyrinth ( J:101670 )

• spaces normally filled with perilymph and endolymph fluids are obliterated with a fibrotic debris

increased cranium width ( J:129632 )

• skull width relative to skull length increase is more pronounced than in Lama2^dy-2J homozygotes