scholarly journals Aminoglycoside-Induced Degeneration of Adult Spiral Ganglion Neurons Involves Differential Modulation of Tyrosine Kinase B and p75 Neurotrophin Receptor Signaling

2006 ◽  
Vol 169 (2) ◽  
pp. 528-543 ◽  
Author(s):  
Justin Tan ◽  
Robert K. Shepherd
Keyword(s):  
Tyrosine Kinase ◽  
Spiral Ganglion ◽  
Neurotrophin Receptor ◽  
Spiral Ganglion Neurons ◽  
P75 Neurotrophin Receptor ◽  
Receptor Signaling ◽  
Differential Modulation ◽  
Ganglion Neurons

Src family tyrosine kinase inhibitors suppress Nav1.1 expression in cultured rat spiral ganglion neurons

2016 ◽  
Vol 202 (3) ◽  
pp. 185-193 ◽  
Author(s):  
Huiying Chen ◽  
Qingjiao Zeng ◽  
Chen Yao ◽  
Zheng Cai ◽  
Tingjia Wei ◽  
...  
Keyword(s):  
Tyrosine Kinase ◽  
Tyrosine Kinase Inhibitors ◽  
Kinase Inhibitors ◽  
Spiral Ganglion ◽  
Spiral Ganglion Neurons ◽  
Ganglion Neurons

scholarly journals ROR1 is essential for proper innervation of auditory hair cells and hearing in humans and mice

2016 ◽  
Vol 113 (21) ◽  
pp. 5993-5998 ◽  
Author(s):  
Oscar Diaz-Horta ◽  
Clemer Abad ◽  
Levent Sennaroglu ◽  
Joseph Foster ◽  
Alexandra DeSmidt ◽  
...  
Keyword(s):  
Tyrosine Kinase ◽  
Inner Ear ◽  
Hair Cells ◽  
Spiral Ganglion ◽  
Auditory Neuropathy ◽  
Mutant Mice ◽  
Spiral Ganglion Neurons ◽  
Type I ◽  
Auditory Hair Cells ◽  
Ganglion Neurons

Hair cells of the inner ear, the mechanosensory receptors, convert sound waves into neural signals that are passed to the brain via the auditory nerve. Little is known about the molecular mechanisms that govern the development of hair cell–neuronal connections. We ascertained a family with autosomal recessive deafness associated with a common cavity inner ear malformation and auditory neuropathy. Via whole-exome sequencing, we identified a variant (c.2207G>C, p.R736T) in ROR1 (receptor tyrosine kinase-like orphan receptor 1), cosegregating with deafness in the family and absent in ethnicity-matched controls. ROR1 is a tyrosine kinase-like receptor localized at the plasma membrane. At the cellular level, the mutation prevents the protein from reaching the cellular membrane. In the presence of WNT5A, a known ROR1 ligand, the mutated ROR1 fails to activate NF-κB. Ror1 is expressed in the inner ear during development at embryonic and postnatal stages. We demonstrate that Ror1 mutant mice are severely deaf, with preserved otoacoustic emissions. Anatomically, mutant mice display malformed cochleae. Axons of spiral ganglion neurons show fasciculation defects. Type I neurons show impaired synapses with inner hair cells, and type II neurons display aberrant projections through the cochlear sensory epithelium. We conclude that Ror1 is crucial for spiral ganglion neurons to innervate auditory hair cells. Impairment of ROR1 function largely affects development of the inner ear and hearing in humans and mice.

TGF-beta superfamily member activin A acts with BDNF and erythropoietin to improve survival of spiral ganglion neurons in vitro

2013 ◽  
Vol 75 ◽  
pp. 416-425 ◽  
Author(s):  
Odett Kaiser ◽  
Gerrit Paasche ◽  
Timo Stöver ◽  
Stefanie Ernst ◽  
Thomas Lenarz ◽  
...  
Keyword(s):  
Spiral Ganglion ◽  
Activin A ◽  
Spiral Ganglion Neurons ◽  
Tgf Beta ◽  
Ganglion Neurons
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