C57BL/6JCya-Tmc1em1flox/Cya
Common Name:
Tmc1-flox
Product ID:
S-CKO-19172
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Tmc1-flox
Strain ID
CKOCMP-13409-Tmc1-B6J-VB
Gene Name
Product ID
S-CKO-19172
Gene Alias
4933416G09Rik; Beethoven; Bth; CWEA1; TMC1ex1; dn
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
19
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Tmc1em1flox/Cya mice (Catalog S-CKO-19172) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000039500
NCBI RefSeq
NM_028953
Target Region
Exon 4
Size of Effective Region
~2.4 kb
Detailed Document
Overview of Gene Research
Tmc1, or transmembrane channel-like protein isoform-1, is a prime candidate for the mechano-electrical transducer (MET) channel in hair cells of the inner ear [1]. It is thought to have a six-transmembrane domain structure and localizes to the tips of the stereocilia in the sensory hair bundle. The function of Tmc1 is crucial for the conversion of mechanical vibrations into electrical signals in the cochlear hair cells, which is fundamental to hearing [3].
In Tmc1 mutant mice, there are significant impacts on auditory function. For example, Tmc1 p.T416K (homologous to a new human deafness mutation) caused deafness and outer hair cell loss by the fourth postnatal week. MET channels in these mutants showed decreased Ca2+ permeability and resting open probability [2]. In Tmc1 p.D569N mutants, by 30 days, mice were profoundly deaf with substantial outer hair cell loss, and MET-channel Ca2+ permeability was reduced, along with reduced expression of the TMC1 D569N channel at the transduction site [5]. In Tmc1;Tmc2 double knockout mice, mechanotransduction currents were lost in hair cells, indicating the involvement of TMC1 in auditory mechanotransduction [3]. LOXHD1 is essential for keeping TMC1-pore forming subunits at the tip link in mouse models, which is important for the function of TMC1-driven mature auditory channels [4].
In conclusion, Tmc1 is essential for the mechano-electrical transduction in cochlear hair cells, playing a key role in the hearing process. Studies using gene knockout mouse models, especially Tmc1 mutants, have revealed its role in maintaining normal auditory function and how its mutations can lead to deafness. Understanding Tmc1 provides insights into the mechanisms of hearing loss and potential therapeutic targets for related diseases.
References:
1. Fettiplace, Robert. . Is TMC1 the Hair Cell Mechanotransducer Channel? In Biophysical journal, 111, 3-9. doi:10.1016/j.bpj.2016.05.032. https://pubmed.ncbi.nlm.nih.gov/27410728/
2. Beurg, Maryline, Schimmenti, Lisa A, Koleilat, Alaa, Ballesteros, Angela, Fettiplace, Robert. 2021. New Tmc1 Deafness Mutations Impact Mechanotransduction in Auditory Hair Cells. In The Journal of neuroscience : the official journal of the Society for Neuroscience, 41, 4378-4391. doi:10.1523/JNEUROSCI.2537-20.2021. https://pubmed.ncbi.nlm.nih.gov/33824189/
3. Deng, Siqi, Yan, Zhiqiang. 2025. TMC1 and TMC2 function as the mechano-electrical transduction ion channel in hearing. In Current opinion in neurobiology, 93, 103026. doi:10.1016/j.conb.2025.103026. https://pubmed.ncbi.nlm.nih.gov/40280017/
4. Wang, Pei, Miller, Katharine K, He, Enqi, Cunningham, Christopher L, Grillet, Nicolas. 2024. LOXHD1 is indispensable for maintaining TMC1 auditory mechanosensitive channels at the site of force transmission. In Nature communications, 15, 7865. doi:10.1038/s41467-024-51850-4. https://pubmed.ncbi.nlm.nih.gov/39256406/
5. Beurg, Maryline, Barlow, Amanda, Furness, David N, Fettiplace, Robert. 2019. A Tmc1 mutation reduces calcium permeability and expression of mechanoelectrical transduction channels in cochlear hair cells. In Proceedings of the National Academy of Sciences of the United States of America, 116, 20743-20749. doi:10.1073/pnas.1908058116. https://pubmed.ncbi.nlm.nih.gov/31548403/
Quality Control Standard
Sperm Test
Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.
Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.
Environmental Standards:SPF
Available Region:Global
Source:Cyagen