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Genetics Home Reference: your guide to understanding genetic conditions
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TECTA

Reviewed February 2016

What is the official name of the TECTA gene?

The official name of this gene is “tectorin alpha.”

TECTA is the gene's official symbol. The TECTA gene is also known by other names, listed below.

What is the normal function of the TECTA gene?

The TECTA gene provides instructions for making a protein called alpha-tectorin. This protein is found in the tectorial membrane, which is part of a snail-shaped structure called the cochlea in the inner ear. The cochlea converts sound waves into nerve impulses, which are then transmitted to the brain. This process is critical for normal hearing.

Alpha-tectorin is large protein with multiple regions (called domains) through which it interacts with other proteins. These interactions are critical for the normal formation of the tectorial membrane.

How are changes in the TECTA gene related to health conditions?

nonsyndromic hearing loss - caused by mutations in the TECTA gene

Researchers have identified at least 40 TECTA gene mutations that can cause nonsyndromic hearing loss, which is loss of hearing that is not associated with other signs and symptoms. Mutations in this gene can cause two forms of nonsyndromic hearing loss: DFNA8/12 and DFNB21.

DFNA8/12 is inherited in an autosomal dominant pattern, which means one mutated copy of the TECTA gene in each cell is sufficient to cause the condition. This form of hearing loss can be present before a child learns to speak (prelingual) or begin after a child learns to speak (postlingual). In some cases the hearing loss is stable, while in others it becomes more severe over time.

The TECTA gene mutations that cause DFNA8/12 change single protein building blocks (amino acids) in alpha-tectorin. The characteristics of the hearing loss depend on the domain in which the mutation occurs. Mutations in one domain tend to affect the ability to hear mid-frequency sounds, while mutations in another generally affect the ability to hear high-frequency sounds. All of these mutations alter the structure of the tectorial membrane and disrupt the conversion of sound to nerve impulses. However, it is unclear why changes in different areas of the alpha-tectorin protein lead to different hearing loss characteristics.

DFNB21 is inherited in an autosomal recessive pattern, which means both copies of the TECTA gene are mutated in each cell. This form of hearing loss is usually severe to profound and is prelingual.

The TECTA gene mutations that cause DFNB21 mutations create a premature stop signal in the instructions for making the alpha-tectorin protein. These mutations lead to the production of a nonfunctional version of alpha-tectorin or prevent cells from making any of this protein. A total loss of alpha-tectorin function alters the structure of the tectorial membrane in such a way that sound cannot be converted to nerve impulses.

Where is the TECTA gene located?

Cytogenetic Location: 11q22-q24

Molecular Location on chromosome 11: base pairs 121,102,666 to 121,190,806

(Homo sapiens Annotation Release 107, GRCh38.p2) (NCBI (http://www.ncbi.nlm.nih.gov/gene/7007))

The TECTA gene is located on the long (q) arm of chromosome 11 between positions 22 and 24.

The TECTA gene is located on the long (q) arm of chromosome 11 between positions 22 and 24.

More precisely, the TECTA gene is located from base pair 121,102,666 to base pair 121,190,806 on chromosome 11.

See How do geneticists indicate the location of a gene? (http://ghr.nlm.nih.gov/handbook/howgeneswork/genelocation) in the Handbook.

Where can I find additional information about TECTA?

You and your healthcare professional may find the following resources about TECTA helpful.

You may also be interested in these resources, which are designed for genetics professionals and researchers.

What other names do people use for the TECTA gene or gene products?

  • DFNA8
  • DFNA12
  • DFNB21
  • TECTA_HUMAN

See How are genetic conditions and genes named? (http://ghr.nlm.nih.gov/handbook/mutationsanddisorders/naming) in the Handbook.

What glossary definitions help with understanding TECTA?

acids ; autosomal ; autosomal dominant ; autosomal recessive ; cell ; cochlea ; domain ; gene ; inherited ; mutation ; postlingual ; prelingual ; protein ; recessive ; tectorial membrane

You may find definitions for these and many other terms in the Genetics Home Reference Glossary.

References

  • Balciuniene J, Dahl N, Jalonen P, Verhoeven K, Van Camp G, Borg E, Pettersson U, Jazin EE. Alpha-tectorin involvement in hearing disabilities: one gene--two phenotypes. Hum Genet. 1999 Sep;105(3):211-6. (http://www.ncbi.nlm.nih.gov/pubmed/10987647?dopt=Abstract)
  • Hildebrand MS, Morín M, Meyer NC, Mayo F, Modamio-Hoybjor S, Mencía A, Olavarrieta L, Morales-Angulo C, Nishimura CJ, Workman H, DeLuca AP, del Castillo I, Taylor KR, Tompkins B, Goodman CW, Schrauwen I, Wesemael MV, Lachlan K, Shearer AE, Braun TA, Huygen PL, Kremer H, Van Camp G, Moreno F, Casavant TL, Smith RJ, Moreno-Pelayo MA. DFNA8/12 caused by TECTA mutations is the most identified subtype of nonsyndromic autosomal dominant hearing loss. Hum Mutat. 2011 Jul;32(7):825-34. doi: 10.1002/humu.21512. Epub 2011 Jun 7. (http://www.ncbi.nlm.nih.gov/pubmed/21520338?dopt=Abstract)
  • Meyer NC, Alasti F, Nishimura CJ, Imanirad P, Kahrizi K, Riazalhosseini Y, Malekpour M, Kochakian N, Jamali P, Van Camp G, Smith RJ, Najmabadi H. Identification of three novel TECTA mutations in Iranian families with autosomal recessive nonsyndromic hearing impairment at the DFNB21 locus. Am J Med Genet A. 2007 Jul 15;143A(14):1623-9. (http://www.ncbi.nlm.nih.gov/pubmed/17431902?dopt=Abstract)
  • Moreno-Pelayo MA, del Castillo I, Villamar M, Romero L, Hernández-Calvín FJ, Herraiz C, Barberá R, Navas C, Moreno F. A cysteine substitution in the zona pellucida domain of alpha-tectorin results in autosomal dominant, postlingual, progressive, mid frequency hearing loss in a Spanish family. J Med Genet. 2001 May;38(5):E13. (http://www.ncbi.nlm.nih.gov/pubmed/11333869?dopt=Abstract)
  • Mustapha M, Weil D, Chardenoux S, Elias S, El-Zir E, Beckmann JS, Loiselet J, Petit C. An alpha-tectorin gene defect causes a newly identified autosomal recessive form of sensorineural pre-lingual non-syndromic deafness, DFNB21. Hum Mol Genet. 1999 Mar;8(3):409-12. (http://www.ncbi.nlm.nih.gov/pubmed/9949200?dopt=Abstract)
  • Naz S, Alasti F, Mowjoodi A, Riazuddin S, Sanati MH, Friedman TB, Griffith AJ, Wilcox ER, Riazuddin S. Distinctive audiometric profile associated with DFNB21 alleles of TECTA. J Med Genet. 2003 May;40(5):360-3. (http://www.ncbi.nlm.nih.gov/pubmed/12746400?dopt=Abstract)
  • NCBI Gene (http://www.ncbi.nlm.nih.gov/gene/7007)
  • Pfister M, Thiele H, Van Camp G, Fransen E, Apaydin F, Aydin O, Leistenschneider P, Devoto M, Zenner HP, Blin N, Nürnberg P, Ozkarakas H, Kupka S. A genotype-phenotype correlation with gender-effect for hearing impairment caused by TECTA mutations. Cell Physiol Biochem. 2004;14(4-6):369-76. (http://www.ncbi.nlm.nih.gov/pubmed/15319541?dopt=Abstract)
  • OMIM: TECTORIN, ALPHA (http://omim.org/entry/602574)
  • Verhoeven K, Van Laer L, Kirschhofer K, Legan PK, Hughes DC, Schatteman I, Verstreken M, Van Hauwe P, Coucke P, Chen A, Smith RJ, Somers T, Offeciers FE, Van de Heyning P, Richardson GP, Wachtler F, Kimberling WJ, Willems PJ, Govaerts PJ, Van Camp G. Mutations in the human alpha-tectorin gene cause autosomal dominant non-syndromic hearing impairment. Nat Genet. 1998 May;19(1):60-2. Erratum in: Nat Genet 1999 Apr;21(4):449. (http://www.ncbi.nlm.nih.gov/pubmed/9590290?dopt=Abstract)

 

The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

 
Reviewed: February 2016
Published: February 8, 2016