Evidence map›Paper›PMID 23348741›Full record

ArticleThe Journal of clinical investigation2013

The LINC complex is essential for hearing.

Henning F Horn, Zippora Brownstein, Danielle R Lenz, Shaked Shivatzki, Amiel A Dror, Orit Dagan-Rosenfeld, Lilach M Friedman, Kyle J Roux, Serguei Kozlov, Kuan-Teh Jeang and 4 more

Open access · greenAbstract read
In one paragraph

Article in The Journal of clinical investigation, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 113 papers.

0numbers the graph read from it
0cells of the map it votes in
113citing papers in PubMed
9.3field-weighted citation impact, top 1% of its field
1 · What the graph read from it

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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

113 citing papers in PubMed, 169 citations in OpenAlex.

  1. Review
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  4. Force-dependent structural dynamics of the giant nesprin-2.Proceedings of the National Academy of Sciences of the United States of America · 2026
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  20. In Vivo Monitoring of Nucleophagy in Caenorhabditis elegans.Methods in molecular biology (Clifton, N.J.) · 2024
    Article

53 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

14 authors at 6 institutions in 3 countries.

Henning F HornInstitute of Medical Biology, A STAR, Singapore.
Zippora Brownstein
Danielle R Lenz
Shaked Shivatzki
Amiel A Dror
Orit Dagan-Rosenfeld
Lilach M Friedman
Kyle J Roux
Serguei Kozlov
Kuan-Teh Jeang
Moshe Frydman
Brian Burke
Colin L Stewart
Karen B Avraham
Tel Aviv University · ILAgency for Science, Technology and Research · SGInstitute of Medical Biology · SGNational Cancer Institute · USNational Institute of Allergy and Infectious Diseases · USSheba Medical Center · IL

Funding

Genomic Approaches to Discovery and Characterization of Genes for Inherited Hearing LossR01DC011835 · NIDCD · TEL AVIV UNIVERSITY · PI AVRAHAM, KAREN B., KANAAN, MOIEN · 2011 to 2022
$3.5M
NIDCD NIH HHS R01 DC011835NIDCD NIH HHS R01DC011835
6 · The paper itself

Abstract

Hereditary hearing loss is the most common sensory deficit. We determined that progressive high-frequency hearing loss in 2 families of Iraqi Jewish ancestry was due to homozygosity for the protein truncating mutation SYNE4 c.228delAT. SYNE4, a gene not previously associated with hearing loss, encodes nesprin-4 (NESP4), an outer nuclear membrane (ONM) protein expressed in the hair cells of the inner ear. The truncated NESP4 encoded by the families' mutation did not localize to the ONM. NESP4 and SUN domain-containing protein 1 (SUN1), which localizes to the inner nuclear membrane (INM), are part of the linker of nucleoskeleton and cytoskeleton (LINC) complex in the nuclear envelope. Mice lacking either Nesp4 or Sun1 were evaluated for hair cell defects and hearing loss. In both Nesp4-/- and Sun1-/- mice, OHCs formed normally, but degenerated as hearing matured, leading to progressive hearing loss. The nuclei of OHCs from mutant mice failed to maintain their basal localization, potentially affecting cell motility and hence the response to sound. These results demonstrate that the LINC complex is essential for viability and normal morphology of OHCs and suggest that the position of the nucleus in sensory epithelial cells is critical for maintenance of normal hearing.

Indexed as

AnimalsDNA Mutational AnalysisFemaleFerrous CompoundsHair Cells, Auditory, OuterHearingHearing Loss, High-FrequencyHumansIraqIsraelJewsMaleMembrane ProteinsMiceMice, Inbred C57BLMice, KnockoutBAE-2FcFerrous CompoundsMembrane ProteinsMultiprotein Complexesnesprin 4 protein, mouseNuclear Proteins

Identifiers

PMID23348741
PMCPMC3561815
OpenAlexW2136651095

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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.