ArticleThe Journal of physiology2008
TRPML3 mutations cause impaired mechano-electrical transduction and depolarization by an inward-rectifier cation current in auditory hair cells of varitint-waddler mice.
Article in The Journal of physiology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
32 citing papers in PubMed, 66 citations in OpenAlex.
- Transient Receptor Potential Channels and Auditory Functions.Antioxidants & redox signaling · 2022Review
- Molecular Structure of the Hair Cell Mechanoelectrical Transduction Complex.Cold Spring Harbor perspectives in medicine · 2019Review
- Are TMCs the Mechanotransduction Channels of Vertebrate Hair Cells?The Journal of neuroscience : the official journal of the Society for Neuroscience · 2016Article
- Article
- The role of TRPMLs in endolysosomal trafficking and function.Cell calcium · 2015Review
- The how and why of identifying the hair cell mechano-electrical transduction channel.Pflugers Archiv : European journal of physiology · 2015Review
- The physiology of mechanoelectrical transduction channels in hearing.Physiological reviews · 2014Review
- HCN1 and HCN2 proteins are expressed in cochlear hair cells: HCN1 can form a ternary complex with protocadherin 15 CD3 and F-actin-binding filamin A or can interact with HCN2.The Journal of biological chemistry · 2012Article
- Constitutive activity of TRPML2 and TRPML3 channels versus activation by low extracellular sodium and small molecules.The Journal of biological chemistry · 2012Article
- Permeation properties of the hair cell mechanotransducer channel provide insight into its molecular structure.Journal of neurophysiology · 2012Article
- TRPC3 and TRPC6 are essential for normal mechanotransduction in subsets of sensory neurons and cochlear hair cells.Open biology · 2012Article
- TRPML: transporters of metals in lysosomes essential for cell survival?Cell calcium · 2011Review
- Expression and vesicular localization of mouse Trpml3 in stria vascularis, hair cells, and vomeronasal and olfactory receptor neurons.The Journal of comparative neurology · 2011Article
- Mucolipin-3 regulates luminal calcium, acidification, and membrane fusion in the endosomal pathway.The Journal of biological chemistry · 2011Article
- Genetic inactivation of Trpml3 does not lead to hearing and vestibular impairment in mice.PloS one · 2010Article
- International Union of Basic and Clinical Pharmacology. LXXVI. Current progress in the mammalian TRP ion channel family.Pharmacological reviews · 2010Review
- Mucolipins: Intracellular TRPML1-3 channels.FEBS letters · 2010Review
- Developmental regulation of TRPC3 ion channel expression in the mouse cochlea.Histochemistry and cell biology · 2010Article
- Opening the TRPML gates.Chemistry & biology · 2010Article
- A quantitative analysis of the spatiotemporal pattern of transient receptor potential gene expression in the developing mouse cochlea.Journal of the Association for Research in Otolaryngology : JARO · 2010Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors at 3 institutions in 2 countries.
Funding
Abstract
TRPML3 (mucolipin-3) belongs to one of the transient-receptor-potential (TRP) ion channel families. Mutations in the Trpml3 gene cause disorganization of the stereociliary hair bundle, structural aberrations in outer and inner hair cells and stria vascularis defects, leading to deafness in the varitint-waddler (Va) mouse. Here we refined the stereociliary localization of TRPML3 and investigated cochlear hair cell function in varitint-waddler (Va(J)) mice carrying the TRPML3<I362T/A419P> mutations. Using a TRPML3-specific antibody we detected a approximately 68 kDa protein with near-equal expression levels in cochlea and vestibule of wild-type and Va(J) mutants. At postnatal days 3 and 5, we observed abundant localization of TRPML3 at the base of stereocilia near the position of the ankle links. This stereociliary localization domain was absent in Va(J) heterozygotes and homozygotes. Electrophysiological recordings revealed reduced mechano-electrical transducer currents in hair cells from Va(J)/+ and Va(J)/Va(J) mice. Furthermore, FM1-43 uptake and [(3)H]gentamicin accumulation were decreased in hair cells in cultured organs of Corti from Va(J)/+ and Va(J)/Va(J) mice. We propose that TRPML3 plays a critical role at the ankle-link region during hair-bundle growth and that an adverse effect of mutant TRPML3 on bundle development and mechano-electrical transduction is the main cause of hearing loss in Va(J)/+ mutant mice. Outer hair cells of Va(J)/Va(J) mice additionally had depolarized resting potentials due to an inwardly rectifying leak conductance formed by the mutant channels, leading over time to hair-cell degeneration and contributing to their deafness. Our findings argue against TRPML3 being a component of the hair-cell transducer channel.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.