Disease relevance of Ear, Inner

• TRPA1, a member of the transient receptor potential (TRP) family of ion channels, is expressed by dorsal root ganglion neurons and by cells of the inner ear, where it has proposed roles in sensing sound, painful cold, and irritating chemicals [1].
• Characteristic associated anomalies include ocular coloboma, choanal atresia, cranial nerve defects, distinctive external and inner ear abnormalities, hearing loss, cardiovascular malformations, urogenital anomalies, and growth retardation [2].
• An X-linked dominant mutation (gyro, gene symbol Gy) in the laboratory mouse causes hypophosphatemia, rickets/osteomalacia, circling behavior, inner ear abnormalities, and sterility in males and a milder phenotype in females [3].
• The high-level expression and stability of cochlin in the inner ear, even in the absence and severe atrophy of the fibrocytes that normally express COCH, are shown through these studies and further elucidate the pathobiologic events occurring in DFNA9 leading to hearing loss and vestibular dysfunction [4].
• Platelet storage pool deficiency associated with inherited abnormalities of the inner ear in the mouse pigment mutants muted and mocha [5].

Psychiatry related information on Ear, Inner

• We examined the spatio/temporal distribution of subtype-specific AMPA- and N-methyl-d-aspartate (NMDA)-selective glutamate receptor proteins in postnatal inner ears from rats during this critical period [6].
• Trks and p75 genes are differentially expressed in the inner ear of human embryos. What may Trks and p75 null mutant mice suggest on human development [7]?

High impact information on Ear, Inner

• Homozygous HOXA1 mutations disrupt human brainstem, inner ear, cardiovascular and cognitive development [8].
• Vascular development in the retina and inner ear: control by Norrin and Frizzled-4, a high-affinity ligand-receptor pair [9].
• In addition, hearing is impaired in the mutant mice at the level of the inner ear, suggesting that urocortin is involved in the normal development of cochlear sensory-cell function [10].
• Myosin-1c (also known as myosin-Ibeta) has been proposed to mediate the slow component of adaptation by hair cells, the sensory cells of the inner ear [11].
• Retinoic acid rescues inner ear defects in Hoxa1 deficient mice [12].

Chemical compound and disease context of Ear, Inner

• Although the symptom of vertigo had been well-recognized for several thousand years, it was not until the pioneering work of Prosper Ménière in the mid-19th century that it was appreciated that vertigo could originate from damage to the inner ear [13].
• These findings will further our understanding of individual GC responsiveness to steroid treatment, and will help improve the development of pharmaceuticals to selectively target GR in the inner ear for individuals with increased sensitivity to acoustic trauma [14].
• We first tested protection of the inner ear in Wistar rats receiving a single infusion of 16 mg CDDP/kg body weight with or without treatment with 100 mg/kg salicylate (bid) for 5 days beginning one day before the CDDP infusion [15].
• The strong interaction between amine groups and PIP2 support the proposal that the latter is crucially involved in aminoglycoside toxicity in the inner ear and kidney [16].
• In particular, the presence of vasopressin receptor mRNA in the inner ear supports the hypothesis of a relationship between high plasma vasopressin levels and endolymphatic hydrops [17].

Biological context of Ear, Inner

• Math1: an essential gene for the generation of inner ear hair cells [18].
• Whereas otic induction proceeds normally in Shh(-/-) embryos, morphogenesis of the inner ear is greatly perturbed by midgestation [19].
• Kinetics of gentamicin uptake and release in the rat. Comparison of inner ear tissues and fluids with other organs [20].
• This article discusses factors important for the development of the inner ear and summarizes cochlear phenotypes of mutant and knockout mice, particularly Otx and Otx2 [21].
• At the early stages of inner ear development, NGF stimulates cell proliferation, an effect mediated through low-affinity receptors [22].

Anatomical context of Ear, Inner

• Stereocilia defects in the sensory hair cells of the inner ear in mice deficient in integrin alpha8beta1 [23].
• Hoxa1 is transiently expressed in the developing hindbrain; its targeted inactivation in mice results in severe abnormalities of the otic capsule and membranous labyrinth [12].
• Here we show that barttin acts as an essential beta-subunit for ClC-Ka and ClC-Kb chloride channels, with which it colocalizes in basolateral membranes of renal tubules and of potassium-secreting epithelia of the inner ear [24].
• This report presents scanning electron micrographs and thin sections of the utricles from the inner ears of guinea pigs that show that, after hair cell loss caused by treatment with the aminoglycoside gentamicin, hair cells reappeared [25].
• This study identifies Sonic hedgehog (Shh) secreted by the notochord and/or floor plate as a primary regulator of auditory cell fates within the mouse inner ear [19].

Associations of Ear, Inner with chemical compounds

• The inner ear Ush1c transcripts predicted several harmonin isoforms, some containing an additional coiled-coil domain and a proline- and serine-rich region [26].
• Recent studies have focused on diverse pathways, including the initiation of actin polymerization, oncogenic tyrosine kinase control of cell transformation, and molecular motor involvement in adaptation of sensory cells of the inner ear [27].
• The authors point out that Prosper Meniere's description of his syndrome (an inner-ear disorder) was not well known when Van Gogh died and that it often was misdiagnosed as epilepsy well into the 20th century [28].
• Inner ear development, although not completely normal, can occur in the absence of TR DNA-binding, suggesting that an alternative and perhaps novel thyroid hormone-signaling pathway may mediate these effects [29].
• Otogelin: a glycoprotein specific to the acellular membranes of the inner ear [30].

Gene context of Ear, Inner

• Here we show that mice homozygous for a targeted disruption of Fgfr3 exhibit skeletal and inner ear defects [31].
• The expression pattern of the murine EYA1 orthologue, Eya1, suggests a role in the development of all components of the inner ear, from the emergence of the otic placode [32].
• Moreover, expression of Gjb3 was identified in rat inner ear tissue by RT-PCR [33].
• The essential function of one of these claudins in the inner ear was established by identifying mutations in CLDN14 that cause nonsyndromic recessive deafness DFNB29 in two large consanguineous Pakistani families [34].
• Moreover, transcripts of DSPP previously reported to be expressed specifically in teeth are also detected in the inner ear of mice [35].

Analytical, diagnostic and therapeutic context of Ear, Inner

• Toward that end, we isolated the corresponding mouse ortholog (Pds) and performed RNA in situ hybridization on mouse inner ears (from 8 days postcoitum to postnatal day 5) to establish the expression pattern of Pds in the developing auditory and vestibular systems [36].
• In this report, we present histopathology, immunohistochemistry and proteomic analyses of inner ear tissues from post-mortem DFNA9 temporal bone samples of an individual from a large Dutch kindred segregating the P51S mutation and adult human unaffected controls, and wild-type (+/+) and Coch null (-/-) knock-out mice [4].
• As assessed by real-time PCR, NOX3 mRNA expression in the inner ear is at least 50-fold higher than in any other tissues where its expression has been observed (e.g. fetal kidney, brain, skull) [37].
• The kinetics of gentamicin in the inner ear fluids of the rat were studied with a sensitive radioimmunoassay [38].
• Intracochlear perfusion and gross potential recording of sound-evoked neural and hair cell responses were used to study the site of action of the L-type Ca(2+) channel blocker nimodipine in the guinea pig inner ear [39].

References