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Tmc1 Gene Detail
Summary
  • Symbol
    Tmc1
  • Name
    transmembrane channel-like gene family 1
  • Synonyms
    4933416G09Rik, Beethoven, Bth
  • Feature Type
    protein coding gene
  • IDs
    MGI:2151016
    NCBI Gene: 13409
  • Alliance
    genome feature page
  • Transcription Start Sites
    1 TSS
Location &
Maps
more
  • Sequence Map
    Chr19:20760822-20931566 bp, - strand
    From Ensembl annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
    Ensembl | UCSC | NCBI
  • Genetic Map
    Chromosome 19, 13.98 cM, cytoband B
  • Mapping Data
    10 experiments
Strain
Comparison
more
  • SNPs within 2kb
    5842 from dbSNP Build 142
  • Strain Annotations
    19
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_2151016
 protein coding gene Chr19:20760820-20931566 (-)
129S1/SvImJ ENSMUSG00200020234
 protein coding gene Chr19:17508179-17679133 (-)
A/J ENSMUSG00195015952
 protein coding gene Chr19:17631825-17803811 (-)
AKR/J ENSMUSG00220010580
 protein coding gene Chr19:17603328-17775075 (-)
BALB/cJ ENSMUSG00180017143
 protein coding gene Chr19:17979965-18152700 (-)
C3H/HeJ ENSMUSG00175003482
 protein coding gene Chr19:17341695-17512661 (-)
C57BL/6NJ ENSMUSG00215016736
 protein coding gene Chr19:17368020-17539070 (-)
CAROLI/EiJ MGP_CAROLIEiJ_G0022812
 protein coding gene Chr19:18040642-18202959 (-)
CAST/EiJ ENSTCUG00005008321
 protein coding gene Chr19:17239778-17410837 (-)
CBA/J ENSMUSG00210012657
 protein coding gene Chr19:17580812-17752757 (-)
DBA/2J ENSMUSG00185013385
 protein coding gene Chr19:17968061-18139019 (-)
FVB/NJ ENSMUSG00205003505
 protein coding gene Chr19:17387579-17559318 (-)
JF1/MsJ ENSUMUG00000017587
 protein coding gene Chr19:18205885-18371534 (-)
LP/J ENSMUSG00230019266
 protein coding gene Chr19:18822594-18993531 (-)
NOD/ShiLtJ ENSMUSG00190019405
 protein coding gene Chr19:17350324-17521273 (-)
NZO/HlLtJ ENSMUSG00225033623
 protein coding gene Chr19:22574841-22740460 (-)
PWK/PhJ ENSLUMG00010008377
 protein coding gene Chr19:17517514-17686362 (-)
SPRET/EiJ ENSMSPG00010018123
 protein coding gene Chr19:17529476-17696179 (-)
WSB/EiJ ENSIUOG00005006877
 protein coding gene Chr19:17074413-17244706 (-)



Homology
more
  • Human Ortholog
    TMC1, transmembrane channel like 1
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    TMC1, transmembrane channel like 1
  • Synonyms
    DFNA36, DFNB11, DFNB7
  • Links
    NCBI Gene ID: 117531
    UniProt: Q8TDI8

  • Chr Location
    9q21.13; chr9:72521608-72838297 (+)  GRCh38
Human Diseases
more
  • Diseases
    2 with Tmc1 mouse models; 3 with human TMC1 associations

Human Disease Mouse Models
      
IDs
 View 3 models
IDs
 View 6 models
      
IDs
Click on a disease name to see all genes associated with that disease.

  • Mutations/Alleles
    5 with disease annotations
  • References
    4 with disease annotations
Mutations,
Alleles, and
Phenotypes
less
  • Phenotype Summary
    27 phenotypes from 6 alleles in 6 genetic backgrounds
    14 phenotypes from multigenic genotypes
    82 phenotype references
Phenotype Overview

adipose tissue
behavior/neurological
cardiovascular system
cellular
craniofacial
digestive/alimentary system
embryo
endocrine/exocrine glands
growth/size/body
hearing/vestibular/ear
hematopoietic system
homeostasis/metabolism
integument
immune system
limbs/digits/tail
liver/biliary system
mortality/aging
muscle
nervous system
pigmentation
renal/urinary system
reproductive system
respiratory system
skeleton
taste/olfaction
neoplasm
vision/eye

Click cells to view annotations.
Mutant mice are characterized by progressive degeneration of the cochlear inner hair cells and concomitant deafness. Different alleles causing progressive deafness or profound congenital deafness.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
    35
  • GO References
    13
Molecular Function

carbohydrate derivative binding
cytoskeletal protein binding
DNA binding
enzyme regulator
hydrolase
ligase
lipid binding
oxidoreductase
RNA binding
signaling receptor activity
signaling receptor binding
transcription
transferase
transporter
Biological Process

carbohydrate derivative metabolism
cell differentiation
cell population proliferation
cellular component organization
DNA-templated transcription
establishment of localization
homeostatic process
immune system process
lipid metabolic process
programmed cell death
protein metabolic process
response to stimulus
signaling
system development
Cellular Component

cell projection
cytoplasmic vesicle
cytoskeleton
cytosol
endoplasmic reticulum
endosome
extracellular region
Golgi apparatus
mitochondrion
membraneless organelle
nucleus
organelle envelope
organelle lumen
plasma membrane
protein-containing complex
synapse
vacuole
Click cells to view annotations.
Expression
less
Expression Overview

early conceptus
embryo ectoderm
embryo endoderm
embryo mesoderm
embryo mesenchyme
extraembryonic component
alimentary system
auditory system
branchial arches
cardiovascular system
connective tissue
endocrine system
exocrine system
hemolymphoid system
integumental system
limbs
liver and biliary system
musculoskeletal system
nervous system
olfactory system
reproductive system
respiratory system
urinary system
visual system
Click cells to view annotations.


  • Assay Results
    754
  • Tissues
    31
  • cDNA Data
    6
  • Literature Summary
    6
  • Comparison Matrix
    Gene Expression + Phenotype
    • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic ENSMUSG00000024749 Ensembl Gene Model | MGI Sequence Detail 170745 C57BL/6J ±  kb
    transcript ENSMUST00000039500 Ensembl | MGI Sequence Detail 4073 Not Applicable  
    polypeptide ENSMUSP00000040859 Ensembl | MGI Sequence Detail 757 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    Molecular
    Reagents
    less
    • All nucleic 9
      Genomic 1
      cDNA 6
      Primer pair 2
      Antibodies 1

      Microarray probesets 3
    Other
    Accession IDs
    less
    MGD-MRK-8873, MGI:1891330, MGI:1921712, MGI:94911
    References
    more
    • Summaries
      All 126
      Developmental Gene Expression 6
      Diseases 4
      Gene Ontology 13
      Phenotypes 82
    • Earliest
      J:236 Deol MS, et al., A new gene for deafness in the mouse. Heredity. 1958;12(4):463-6
    • Latest
      J:386576 George SS, et al., Auditory Hair Cell Mechanotransduction Channels Dynamically Shape the Mechanical Properties of Their Membrane Environment. Adv Sci (Weinh). 2026 Feb;13(7):e08268
    Contributing Projects:
    Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
    Citing These Resources
    Funding Information
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    Send questions and comments to User Support.     		last database update
    09/01/2026
    MGI 6.24     		The Jackson Laboratory