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TECTA gene
URL of this page: https://medlineplus.gov/genetics/gene/tecta/

TECTA gene

tectorin alpha

Normal Function

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 electrical nerve impulses, which are then transmitted to the brain. This process is critical for normal hearing.

Alpha-tectorin is a large protein with multiple regions (called domains) through which it interacts with itself and several other proteins. These interactions help the tectorial membrane develop properly.

Health Conditions Related to Genetic Changes

Nonsyndromic hearing loss

Genetic changes that cause disease are called pathogenic variants. Pathogenic variants in the TECTA gene have been found to cause nonsyndromic hearing loss, which is a partial or total loss of hearing that is not associated with other signs and symptoms. Variants 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 altered copy of the TECTA gene in each cell is sufficient to cause the condition. DFNA8/12 can be present before a child learns to speak (prelingual) or begin after a child learns to speak (postlingual).

The pathogenic variants in the TECTA gene that cause DFNA8/12 lead to the substitution of one protein building block (amino acid) for another in alpha-tectorin. The characteristics of the hearing loss depend on the domain in which the amino acid substitution occurs. Changes in one domain can affect the ability to hear mid-frequency sounds, while changes in another may affect the ability to hear high-frequency sounds. All of these changes alter the structure of the tectorial membrane and disrupt the conversion of sound to nerve impulses. However, it is unclear why alterations in different domains of the alpha-tectorin protein lead to different types of hearing loss.

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

The pathogenic variants that cause DFNB21 create a premature stop signal in the instructions for making the alpha-tectorin protein. As a result, cells produce a nonfunctional version of alpha-tectorin or do not make any protein at all. A total loss of functional alpha-tectorin prevents the formation of a working tectorial membrane. In these cases, sound cannot be converted to nerve impulses, leading to nonsyndromic hearing loss.

More About This Health Condition

Other Names for This Gene

  • DFNA12
  • DFNA8
  • DFNB21

Additional Information & Resources

Tests Listed in the Genetic Testing Registry

Scientific Articles on PubMed

Catalog of Genes and Diseases from OMIM

Gene and Variant Databases

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. doi: 10.1007/s004390051091. Citation on PubMed
  • Gonzalez-Aguado R, Onecha E, Gallo-Teran J, Morales-Angulo C. Hearing Loss Secondary to TECTA Gene Mutations. Ann Otol Rhinol Laryngol. 2025 Jun;134(6):405-413. doi: 10.1177/00034894251315550. Epub 2025 Feb 4. Citation on PubMed
  • Hildebrand MS, Morin M, Meyer NC, Mayo F, Modamio-Hoybjor S, Mencia 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. Citation on PubMed or Free article on PubMed Central
  • 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. doi: 10.1002/ajmg.a.31718. Citation on PubMed
  • Moreno-Pelayo MA, del Castillo I, Villamar M, Romero L, Hernandez-Calvin FJ, Herraiz C, Barbera 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. doi: 10.1136/jmg.38.5.e13. No abstract available. Citation on PubMed or Free article on PubMed Central
  • 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. doi: 10.1093/hmg/8.3.409. Citation on PubMed
  • 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. doi: 10.1136/jmg.40.5.360. No abstract available. Citation on PubMed or Free article on PubMed Central
  • Pfister M, Thiele H, Van Camp G, Fransen E, Apaydin F, Aydin O, Leistenschneider P, Devoto M, Zenner HP, Blin N, Nurnberg 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. doi: 10.1159/000080347. Citation on PubMed
  • 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. doi: 10.1038/ng0598-60. Citation on PubMed
  • Yang Y, Xiong Y, Lai H, Feng C, Chen Z, Huang Y, Guo Z, Li X, Luo L, Zhao F, Wu P, Luo H, Liu Y, Liu Y, Zou Y. A novel splicing variant in TECTA associated with prelingual autosomal dominant nonsyndromic hearing loss via dominant-negative effect. Hum Mol Genet. 2025 Sep 3;34(18):1517-1525. doi: 10.1093/hmg/ddaf109. Citation on PubMed

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