A compendium of mouse knockouts with inner ear defects (Q50486350)

14 March 2018

title

A compendium of mouse knockouts with inner ear defects (English)

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14 March 2018

author name string

Anna V Anagnostopoulos

series ordinal

1

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14 March 2018

publication date

1 October 2002

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14 March 2018

published in

Trends in Genetics

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14 March 2018

volume

18

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14 March 2018

issue

10

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14 March 2018

page(s)

499

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14 March 2018

Development of the mouse inner ear and origin of its sensory organs.

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Hair cell function - it's all a matter of organization

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ENU mutagenesis and the search for deafness genes

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A systematic, genome-wide, phenotype-driven mutagenesis programme for gene function studies in the mouse

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Mouse mutants from chemically mutagenized embryonic stem cells

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Paradigms and paradoxes: mouse (and human) models of genetic deafness.

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Growth factor regulation of the cell cycle in developing and mature inner ear sensory epithelia

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Development of the vertebrate ear: insights from knockouts and mutants

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Patterning of the mammalian cochlea

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Genetic patterning of embryonic inner ear development.

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Auditory and vestibular mouse mutants: models for human deafness

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Molecular genetics of hearing loss

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7 January 2021

Retinoic acid rescues inner ear defects in Hoxa1 deficient mice.

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7 January 2021

Developmental defects of the ear, cranial nerves and hindbrain resulting from targeted disruption of the mouse homeobox gene Hox-1.6

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7 January 2021

Disruption of the Hox-1.6 homeobox gene results in defects in a region corresponding to its rostral domain of expression

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7 January 2021

Altered segmental identity and abnormal migration of motor neurons in mice lacking Hoxb-1.

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7 January 2021

Hoxa-2 mutant mice exhibit homeotic transformation of skeletal elements derived from cranial neural crest

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A homeotic transformation is generated in the rostral branchial region of the head by disruption of Hoxa-2, which acts as a selector gene

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7 January 2021

Retinoid X receptors are essential for early mouse development and placentogenesis

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7 January 2021

Specific expression of the retinoic acid-synthesizing enzyme RALDH2 during mouse inner ear development

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7 January 2021

The winged helix transcription factor Fkh10 is required for normal development of the inner ear.

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7 January 2021

A human homologue of the Drosophila eyes absent gene underlies branchio-oto-renal (BOR) syndrome and identifies a novel gene family

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7 January 2021

Inner ear and kidney anomalies caused by IAP insertion in an intron of the Eya1 gene in a mouse model of BOR syndrome

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7 January 2021

Eya1-deficient mice lack ears and kidneys and show abnormal apoptosis of organ primordia

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7 January 2021

Transcription factor AP-2 essential for cranial closure and craniofacial development

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7 January 2021

Neural tube, skeletal and body wall defects in mice lacking transcription factor AP-2

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7 January 2021

The role of FGF-3 in early inner ear development: an analysis in normal and kreisler mutant mice

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7 January 2021

The mouse segmentation gene kr encodes a novel basic domain-leucine zipper transcription factor

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7 January 2021

Skeletal overgrowth and deafness in mice lacking fibroblast growth factor receptor 3.

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7 January 2021

Role for basic fibroblast growth factor (FGF-2) in tyrosine kinase (TrkB) expression in the early development and innervation of the auditory receptor: in vitro and in situ studies.

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7 January 2021

FGF10 acts as a major ligand for FGF receptor 2 IIIb in mouse multi-organ development

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7 January 2021

Fgf-10 is required for both limb and lung development and exhibits striking functional similarity to Drosophila branchless

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7 January 2021

Fgf10 is essential for limb and lung formation

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7 January 2021

Fgfr2 is required for limb outgrowth and lung-branching morphogenesis

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7 January 2021

FGF/FGFR-2(IIIb) signaling is essential for inner ear morphogenesis

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7 January 2021

FGFs control the patterning of the inner ear but are not able to induce the full ear program

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7 January 2021

Expression of GATA-3 during lymphocyte differentiation and mouse embryogenesis

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7 January 2021

Transcription factor GATA-3 alters pathway selection of olivocochlear neurons and affects morphogenesis of the ear

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7 January 2021

Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness.

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7 January 2021

The sex-linked fidget mutation abolishes Brn4/Pou3f4 gene expression in the embryonic inner ear

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7 January 2021

The paired-like homeo box gene MHox is required for early events of skeletogenesis in multiple lineages

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7 January 2021

Epilepsy and brain abnormalities in mice lacking the Otx1 gene

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7 January 2021

Mouse Otx2 functions in the formation and patterning of rostral head.

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7 January 2021

Role of the Dlx homeobox genes in proximodistal patterning of the branchial arches: mutations of Dlx-1, Dlx-2, and Dlx-1 and -2 alter morphogenesis of proximal skeletal and soft tissue structures derived from the first and second arches

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7 January 2021

Cloning and characterization of two members of the vertebrate Dlx gene family

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7 January 2021

Differential and overlapping expression domains of Dlx-2 and Dlx-3 suggest distinct roles for Distal-less homeobox genes in craniofacial development

1 reference

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7 January 2021

The nuclear receptor Nor-1 is essential for proliferation of the semicircular canals of the mouse inner ear

1 reference

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7 January 2021

Normal development, growth and reproduction in cellular retinoic acid binding protein-I (CRABPI) null mutant mice

1 reference

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7 January 2021

Spatio-temporal distribution of cellular retinoid binding protein gene transcripts in the developing and the adult cochlea. Morphological and functional consequences in CRABP- and CRBPI-null mutant mice.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Math1 is essential for genesis of cerebellar granule neurons

1 reference

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7 January 2021

Math1: an essential gene for the generation of inner ear hair cells

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7 January 2021

Proprioceptor pathway development is dependent on Math1.

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7 January 2021

Targeted disruption of mammalian hairy and Enhancer of split homolog-1 (HES-1) leads to up-regulation of neural helix-loop-helix factors, premature neurogenesis, and severe neural tube defects

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7 January 2021

Hes1 and Hes5 activities are required for the normal development of the hair cells in the mammalian inner ear

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https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Hair cells and supporting cells share a common progenitor in the avian inner ear.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

A mutation in the Lunatic fringe gene suppresses the effects of a Jagged2 mutation on inner hair cell development in the cochlea

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7 January 2021

Notch signaling: cell fate control and signal integration in development

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7 January 2021

Hes1 and Hes5 as notch effectors in mammalian neuronal differentiation.

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7 January 2021

Notch signalling pathway mediates hair cell development in mammalian cochlea

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7 January 2021

Embryonic lethality and vascular defects in mice lacking the Notch ligand Jagged1

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7 January 2021

Notch1 is essential for postimplantation development in mice.

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7 January 2021

Defects in limb, craniofacial, and thymic development in Jagged2 mutant mice

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7 January 2021

Defects in somite formation in lunatic fringe-deficient mice

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7 January 2021

Role of transcription factors Brn-3.1 and Brn-3.2 in auditory and visual system development

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7 January 2021

Essential role of POU-domain factor Brn-3c in auditory and vestibular hair cell development

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7 January 2021

Effects of a hair cell transcription factor, Brn-3.1, gene deletion on homozygous and heterozygous mouse cochleas in adulthood and aging

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7 January 2021

Mutation in transcription factor POU4F3 associated with inherited progressive hearing loss in humans

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7 January 2021

Chondrodysplasia and neurological abnormalities in ATF-2-deficient mice

1 reference

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7 January 2021

Stereocilia defects in the sensory hair cells of the inner ear in mice deficient in integrin alpha8beta1

1 reference

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7 January 2021

Apaf1 (CED-4 homolog) regulates programmed cell death in mammalian development

1 reference

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7 January 2021

Adult Apaf-1-deficient mice exhibit male infertility

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7 January 2021

Apaf1 in developmental apoptosis and cancer: how many ways to die?

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7 January 2021

Decreased apoptosis in the brain and premature lethality in CPP32-deficient mice

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7 January 2021

Deafness due to degeneration of cochlear neurons in caspase-3-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Caspase-3-deficiency induces hyperplasia of supporting cells and degeneration of sensory cells resulting in the hearing loss

1 reference

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7 January 2021

A syndrome of multiorgan hyperplasia with features of gigantism, tumorigenesis, and female sterility in p27(Kip1)-deficient mice

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7 January 2021

Gene disruption of p27(Kip1) allows cell proliferation in the postnatal and adult organ of corti

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7 January 2021

Thyroid disease and hearing disorders: new genetic links.

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7 January 2021

Abnormal heart rate and body temperature in mice lacking thyroid hormone receptor alpha 1

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https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Thyroid hormone receptor beta-dependent expression of a potassium conductance in inner hair cells at the onset of hearing

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https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Genetic analysis reveals different functions for the products of the thyroid hormone receptor alpha locus

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Suppression of the deafness and thyroid dysfunction in Thrb-null mice by an independent mutation in the Thra thyroid hormone receptor alpha gene

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Thyroid hormone receptor beta is essential for development of auditory function

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Divergent roles for thyroid hormone receptor beta isoforms in the endocrine axis and auditory system

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Retardation of cochlear maturation and impaired hair cell function caused by deletion of all known thyroid hormone receptors.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

The Protective Effects of Tirilated Mesylate (U74006F) on Ischemic and Reperfusion-Induced Cochlear Damage

1 reference

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7 January 2021

The protective effects of allopurinol and superoxide dismutase on noise-induced cochlear damage.

1 reference

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7 January 2021

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Targeted deletion of the cytosolic Cu/Zn-superoxide dismutase gene (Sod1) increases susceptibility to noise-induced hearing loss

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Targeted mutation of the gene for cellular glutathione peroxidase (Gpx1) increases noise-induced hearing loss in mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Lack of calbindin-D28k does not affect hearing level or survival of hair cells in acoustic trauma.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Mechano-electrical transduction in mice lacking the alpha-subunit of the epithelial sodium channel

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Congenital deafness and sinoatrial node dysfunction in mice lacking class D L-type Ca2+ channels

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

A targeted deletion in alpha-tectorin reveals that the tectorial membrane is required for the gain and timing of cochlear feedback.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Mutations in the human alpha-tectorin gene cause autosomal dominant non-syndromic hearing impairment

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Targeted disruption of otog results in deafness and severe imbalance

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Mutations in COL11A2 cause non-syndromic hearing loss (DFNA13)

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Ultrastructural, physiological, and molecular defects in the inner ear of a gene-knockout mouse model for autosomal Alport syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Immunolocalization of ClC-K chloride channel in strial marginal cells and vestibular dark cells

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

An ATP-dependent inwardly rectifying potassium channel, KAB-2 (Kir4. 1), in cochlear stria vascularis of inner ear: its specific subcellular localization and correlation with the formation of endocochlear potential.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Mechanism generating endocochlear potential: role played by intermediate cells in stria vascularis

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

KCNJ10 (Kir4.1) potassium channel knockout abolishes endocochlear potential

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

KCNQ1/KCNE1 potassium channels in mammalian vestibular dark cells

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Targeted disruption of the Kvlqt1 gene causes deafness and gastric hyperplasia in mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Targeted disruption of the Kcnq1 gene produces a mouse model of Jervell and Lange-Nielsen Syndrome.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Inner ear defects induced by null mutation of the isk gene

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Involvement of IsK-associated K+ channel in heart rate control of repolarization in a murine engineered model of Jervell and Lange-Nielsen syndrome

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Deafness and imbalance associated with inactivation of the secretory Na-K-2Cl co-transporter

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Mice lacking the basolateral Na-K-2Cl cotransporter have impaired epithelial chloride secretion and are profoundly deaf.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Failure of spermatogenesis in mouse lines deficient in the Na(+)-K(+)-2Cl(-) cotransporter

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Ultrastructure of the inner ear of NKCC1-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Balance and hearing deficits in mice with a null mutation in the gene encoding plasma membrane Ca2+-ATPase isoform 2

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https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Mutations in a plasma membrane Ca2+-ATPase gene cause deafness in deafwaddler mice

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https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Effects of PMCA2 mutation on DPOAE amplitudes and latencies in deafwaddler mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Generation and phenotype of a transgenic knockout mouse lacking the mercurial-insensitive water channel aquaporin-4.

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https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Impaired hearing in mice lacking aquaporin-4 water channels

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https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Ephrins and their Eph receptors: multitalented directors of embryonic development

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https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

EphB2 guides axons at the midline and is necessary for normal vestibular function

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Sound needs sound melanocytes to be heard

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Targeted and natural (piebald-lethal) mutations of endothelin-B receptor gene produce megacolon associated with spotted coat color in mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Interaction of endothelin-3 with endothelin-B receptor is essential for development of epidermal melanocytes and enteric neurons

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Gap junctions in the rat cochlea: immunohistochemical and ultrastructural analysis

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

KCNQ4, a K+ channel mutated in a form of dominant deafness, is expressed in the inner ear and the central auditory pathway.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter Kcc4.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Intracellular anions as the voltage sensor of prestin, the outer hair cell motor protein

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Transplacental uptake of glucose is decreased in embryonic lethal connexin26-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Connexin31-deficiency in mice causes transient placental dysmorphogenesis but does not impair hearing and skin differentiation.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Lineages and transcription factors in the specification of vertebrate primary sensory neurons

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Neurogenin 1 null mutant ears develop fewer, morphologically normal hair cells in smaller sensory epithelia devoid of innervation

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Diabetes, defective pancreatic morphogenesis, and abnormal enteroendocrine differentiation in BETA2/neuroD-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

NeuroD is required for differentiation of the granule cells in the cerebellum and hippocampus

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Essential role of BETA2/NeuroD1 in development of the vestibular and auditory systems

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

The combined effects of trkB and trkC mutations on the innervation of the inner ear.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Severe sensory and sympathetic deficits in mice lacking neurotrophin-3.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Effects of neurotrophin and neurotrophin receptor disruption on the afferent inner ear innervation.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Severe sensory deficits but normal CNS development in newborn mice lacking TrkB and TrkC tyrosine protein kinase receptors

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Survival of inner ear sensory neurons in trk mutant mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

NGF, BDNF and NT-3 play unique roles in the in vitro development and patterning of innervation of the mammalian inner ear

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Targeted deletion of the mouse POU domain gene Brn-3a causes selective loss of neurons in the brainstem and trigeminal ganglion, uncoordinated limb movement, and impaired suckling

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Delayed inner ear maturation and neuronal loss in postnatal Igf-1-deficient mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Role of alpha9 nicotinic ACh receptor subunits in the development and function of cochlear efferent innervation

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Tenascin-C in the cochlea of the developing mouse.

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Abnormal behavior and neurotransmissions of tenascin gene knockout mouse

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophy

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Metachromatic leukodystrophy: molecular genetics and an animal model

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Hyperactivity, neuromotor defects, and impaired learning and memory in a mouse model for metachromatic leukodystrophy

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Progressive cerebellar, auditory, and esophageal dysfunction caused by targeted disruption of the frizzled-4 gene

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Disruption of mdr1a p-glycoprotein gene results in dysfunction of blood-inner ear barrier in mice

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Cyclosporin A inhibits the extrusion pump function of p-glycoprotein in the inner ear of mice treated with vinblastine and doxorubicin

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Haploinsufficient phenotypes in Bmp4 heterozygous null mice and modification by mutations in Gli3 and Alx4

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

An animal model for Norrie disease (ND): gene targeting of the mouse ND gene

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Unrestrained nociceptive response and disregulation of hearing ability in mice lacking the nociceptin/orphaninFQ receptor

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

Retinal degeneration but not obesity is observed in null mutants of the tubby-like protein 1 gene

1 reference

https://api.crossref.org/works/10.1016%2FS0168-9525%2802%2902753-1

7 January 2021

10.1016/S0168-9525(02)02753-1

Identifiers

DOI

1 reference

14 March 2018

1 reference

14 March 2018