Carcinoembryonic antigen-related cell adhesion molecule 16 interacts with alpha-tectorin and is mutated in autosomal dominant hearing loss (DFNA4)

Jing Zheng; Katharine K Miller; Tao Yang; Michael S Hildebrand; A Eliot Shearer; Adam P DeLuca; Todd E Scheetz; Jennifer Drummond; Steve E Scherer; P Kevin Legan; Richard J Goodyear; Guy P Richardson; Mary Ann Cheatham; Richard J Smith; Peter Dallos

doi:10.1073/pnas.1005842108

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Carcinoembryonic antigen-related cell adhesion molecule 16 interacts with alpha-tectorin and is mutated in autosomal dominant hearing loss (DFNA4)

Journal article

Open access

Peer reviewed

Carcinoembryonic antigen-related cell adhesion molecule 16 interacts with alpha-tectorin and is mutated in autosomal dominant hearing loss (DFNA4)

Jing Zheng, Katharine K Miller, Tao Yang, Michael S Hildebrand, A Eliot Shearer, Adam P DeLuca, Todd E Scheetz, Jennifer Drummond, Steve E Scherer, P Kevin Legan, …

Proceedings of the National Academy of Sciences - PNAS, Vol.108(10), pp.4218-4223

03/08/2011

DOI: 10.1073/pnas.1005842108

PMCID: PMC3054008

PMID: 21368133

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Abstract

We report on a secreted protein found in mammalian cochlear outer hair cells (OHC) that is a member of the carcinoembryonic antigen-related cell adhesion molecule (CEACAM) family of adhesion proteins. Ceacam16 mRNA is expressed in OHC, and its protein product localizes to the tips of the tallest stereocilia and the tectorial membrane (TM). This specific localization suggests a role in maintaining the integrity of the TM as well as in the connection between the OHC stereocilia and TM, a linkage essential for mechanical amplification. In agreement with this role, CEACAM16 colocalizes and coimmunoprecipitates with the TM protein α-tectorin. In addition, we show that mutation of CEACAM16 leads to autosomal dominant nonsyndromic deafness (ADNSHL) at the autosomal dominant hearing loss (DFNA4) locus. In aggregate, these data identify CEACAM16 as an α-tectorin-interacting protein that concentrates at the point of attachment of the TM to the stereocilia and, when mutated, results in ADNSHL at the DFNA4 locus.

Mutation

Cell Adhesion Molecules - chemistry

Amino Acid Sequence

Cell Adhesion Molecules - genetics

Humans

RNA, Messenger - genetics

Molecular Sequence Data

Myosin Heavy Chains - genetics

Cell Adhesion Molecules - metabolism

Blotting, Western

GPI-Linked Proteins - metabolism

Hearing Loss - genetics

Hearing Loss - metabolism

Animals

In Situ Hybridization

Genes, Dominant

Mice

Myosin Type II - genetics

Extracellular Matrix Proteins - metabolism

Details

Title: Subtitle: Carcinoembryonic antigen-related cell adhesion molecule 16 interacts with alpha-tectorin and is mutated in autosomal dominant hearing loss (DFNA4)
Creators: Jing Zheng - Northwestern University
Katharine K Miller
Tao Yang - University of Iowa
Michael S Hildebrand
A Eliot Shearer
Adam P DeLuca
Todd E Scheetz
Jennifer Drummond
Steve E Scherer
P Kevin Legan
Richard J Goodyear
Guy P Richardson
Mary Ann Cheatham
Richard J Smith
Peter Dallos
Resource Type: Journal article
Publication Details: Proceedings of the National Academy of Sciences - PNAS, Vol.108(10), pp.4218-4223
DOI: 10.1073/pnas.1005842108
PMID: 21368133
PMCID: PMC3054008
NLM abbreviation: Proc Natl Acad Sci U S A
ISSN: 0027-8424
eISSN: 1091-6490
Publisher: United States
Grant note: Wellcome Trust DC00089 / NIDCD NIH HHS UL1 RR025741 / NCRR NIH HHS T32 GM007337 / NIGMS NIH HHS R01 DC002842 / NIDCD NIH HHS R01 DC006412 / NIDCD NIH HHS DCO3544 / PHS HHS 087737 / Wellcome Trust DC010633 / NIDCD NIH HHS R01 DC003544 / NIDCD NIH HHS DC006412 / NIDCD NIH HHS RC1 DC010633 / NIDCD NIH HHS R01 DC000089 / NIDCD NIH HHS
Language: English
Date published: 03/08/2011
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Electrical and Computer Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Center for Bioinformatics and Computational Biology; Otolaryngology; Internal Medicine; Ophthalmology and Visual Sciences
Record Identifier: 9983979981102771

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