Gata3 is required for the functional maturation of inner hair cells and their innervation in the mouse cochlea.
Animals
Cell Differentiation
/ physiology
Cochlea
/ metabolism
GATA3 Transcription Factor
/ metabolism
Hair Cells, Auditory, Inner
/ metabolism
Hair Cells, Auditory, Outer
/ metabolism
Hair Cells, Vestibular
/ metabolism
Hearing
/ physiology
Hearing Loss
/ metabolism
Membrane Proteins
/ metabolism
Mice, Knockout
Mice, Transgenic
Sensory Receptor Cells
/ metabolism
Synapses
/ metabolism
Deafness
Development
Gata3
Hair cells
Hypoprathyroidism, Deafness and Real anomaly Syndrome
afferent fibre
cochlea
Journal
The Journal of physiology
ISSN: 1469-7793
Titre abrégé: J Physiol
Pays: England
ID NLM: 0266262
Informations de publication
Date de publication:
07 2019
07 2019
Historique:
received:
14
03
2019
accepted:
07
05
2019
pubmed:
10
5
2019
medline:
28
7
2020
entrez:
10
5
2019
Statut:
ppublish
Résumé
The physiological maturation of auditory hair cells and their innervation requires precise temporal and spatial control of cell differentiation. The transcription factor gata3 is essential for the earliest stages of auditory system development and for survival and synaptogenesis in auditory sensory afferent neurons. We show that during postnatal development in the mouse inner ear gata3 is required for the biophysical maturation, growth and innervation of inner hair cells; in contrast, it is required only for the survival of outer hair cells. Loss of gata3 in inner hair cells causes progressive hearing loss and accounts for at least some of the deafness associated with the human hypoparathyroidism, deafness and renal anomaly (HDR) syndrome. The results show that gata3 is critical for later stages of mammalian auditory system development where it plays distinct, complementary roles in the coordinated maturation of sensory hair cells and their innervation. The zinc finger transcription factor gata3 regulates inner ear development from the formation of the embryonic otic placode. Throughout development, gata3 is expressed dynamically in all the major cochlear cell types. Its role in afferent formation is well established but its possible involvement in hair cell maturation remains unknown. Here, we find that in heterozygous gata3 null mice (gata3
Identifiants
pubmed: 31069810
doi: 10.1113/JP277997
pmc: PMC6636704
doi:
Substances chimiques
GATA3 Transcription Factor
0
Gata3 protein, mouse
0
Membrane Proteins
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
3389-3406Subventions
Organisme : Wellcome Trust
ID : 102892/Z/13/Z
Pays : United Kingdom
Organisme : Action on Hearing Loss
ID : 562:SHE:MH
Pays : United Kingdom
Organisme : Wellcome Trust
ID : WT102892MA
Pays : United Kingdom
Informations de copyright
© 2019 The Authors. The Journal of Physiology published by John Wiley & Sons Ltd on behalf of The Physiological Society.
Références
Cell. 1999 Feb 5;96(3):437-46
pubmed: 10025409
Nat Genet. 1999 Apr;21(4):363-9
pubmed: 10192385
Dev Biol. 1999 Jun 15;210(2):305-21
pubmed: 10357893
Eur J Immunol. 1999 Jun;29(6):1912-8
pubmed: 10382753
J Neurocytol. 1998 Sep;27(9):637-47
pubmed: 10447238
J Physiol. 1999 Nov 1;520 Pt 3:653-60
pubmed: 10545133
Pflugers Arch. 1999 Dec;439(1-2):113-22
pubmed: 10651007
Nat Genet. 2000 Jun;25(2):209-12
pubmed: 10835639
Science. 2000 Jun 30;288(5475):2366-8
pubmed: 10875922
Neuron. 2000 Jun;26(3):595-601
pubmed: 10896156
J Neurosci. 2000 Aug 15;20(16):6125-34
pubmed: 10934262
Nature. 2000 Jul 27;406(6794):419-22
pubmed: 10935639
J Comp Neurol. 2001 Jan 22;429(4):615-30
pubmed: 11135239
J Med Genet. 2001 Jun;38(6):374-80
pubmed: 11389161
J Comp Neurol. 2002 Jan 21;442(4):378-91
pubmed: 11793341
J Physiol. 2003 Apr 15;548(Pt 2):383-400
pubmed: 12588897
Mech Dev. 2004 Mar;121(3):287-99
pubmed: 15003631
J Physiol. 2004 Jun 1;557(Pt 2):613-33
pubmed: 15064328
Neurobiol Dis. 2004 Jun;16(1):169-78
pubmed: 15207274
J Physiol. 2004 Nov 1;560(Pt 3):691-708
pubmed: 15331671
J Neurosci. 2004 Sep 8;24(36):7814-20
pubmed: 15356192
Nat Immunol. 2004 Nov;5(11):1157-65
pubmed: 15475959
Neurobiol Dis. 2005 Dec;20(3):890-7
pubmed: 15994092
Development. 2006 Aug;133(15):2817-26
pubmed: 16790479
Nature. 2006 Jun 22;441(7096):984-7
pubmed: 16791196
Mech Dev. 2006 Jun;123(6):415-29
pubmed: 16806848
Development. 2007 Jan;134(2):261-72
pubmed: 17151017
Stem Cells Dev. 2007 Aug;16(4):605-13
pubmed: 17784834
Hear Res. 1991 Jun;53(2):293-311
pubmed: 1880082
PLoS One. 2009 Sep 23;4(9):e7144
pubmed: 19774072
Cell Stem Cell. 2009 Oct 2;5(4):420-33
pubmed: 19796622
Dev Biol. 2010 Jan 1;337(1):134-46
pubmed: 19854167
Oncogene. 2010 May 6;29(18):2739-45
pubmed: 20154722
Prog Neurobiol. 2011 Apr;93(4):488-508
pubmed: 21232575
Int J Dev Biol. 2011;55(3):297-303
pubmed: 21553382
Hear Res. 2012 Jan;283(1-2):151-61
pubmed: 22094003
J Neurosci. 2012 Jun 20;32(25):8545-53
pubmed: 22723694
Dev Biol. 2013 Feb 1;374(1):210-22
pubmed: 23220102
PLoS One. 2013 Apr 16;8(4):e62046
pubmed: 23614009
Proc Natl Acad Sci U S A. 2013 May 21;110(21):8720-5
pubmed: 23650376
Hum Mol Genet. 2013 Sep 15;22(18):3609-23
pubmed: 23666531
Dev Biol. 2013 Oct 15;382(2):555-66
pubmed: 23920117
Elife. 2013 Dec 10;2:e01341
pubmed: 24327562
Audiol Neurootol. 2014;19(2):106-14
pubmed: 24434941
Proc Natl Acad Sci U S A. 2014 Oct 14;111(41):14918-23
pubmed: 25228765
Curr Opin Genet Dev. 2015 Jun;32:112-8
pubmed: 25796080
Development. 2015 May 1;142(9):1561-71
pubmed: 25922522
J Anat. 2016 Feb;228(2):233-54
pubmed: 26052920
J Neurosci. 2016 Jan 13;36(2):336-49
pubmed: 26758827
J Physiol. 2016 Jul 1;594(13):3667-81
pubmed: 27111754
J Neurosci. 2017 Mar 29;37(13):3447-3464
pubmed: 28209736
Mol Pharmacol. 2017 Oct;92(4):375-388
pubmed: 28874607
Front Mol Neurosci. 2017 Oct 18;10:326
pubmed: 29093662
Nat Commun. 2018 Oct 1;9(1):4015
pubmed: 30275467
Genet Mol Biol. 2018 Oct-Dec;41(4):794-798
pubmed: 30534854
EMBO J. 2019 May 2;38(9):null
pubmed: 30804003
J Neurocytol. 1986 Dec;15(6):693-714
pubmed: 3819777
J Physiol. 1971 Feb;212(3):739-61
pubmed: 5557069
Nat Genet. 1995 Sep;11(1):40-4
pubmed: 7550312
Development. 1993 Dec;119(4):1041-53
pubmed: 8306874
J Comp Neurol. 1996 Jul 08;370(4):551-62
pubmed: 8807454
Nature. 1998 Jul 16;394(6690):281-4
pubmed: 9685158