Tmc1 Antibody

Code CSB-PA840294XA01MO
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Product Details

Full Product Name
Rabbit anti-Mus musculus (Mouse) Tmc1 Polyclonal antibody
Uniprot No.
Target Names
Tmc1
Alternative Names
Tmc1; Bth; dn; Transmembrane channel-like protein 1; Beethoven protein; Deafness protein; Transmembrane cochlear-expressed protein 1
Raised in
Rabbit
Species Reactivity
Mus musculus (Mouse)
Immunogen
Recombinant Mus musculus (Mouse) Tmc1 protein
Immunogen Species
Mus musculus (Mouse)
Conjugate
Non-conjugated
Clonality
Polyclonal
Isotype
IgG
Purification Method
Antigen Affinity Purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, WB (ensure identification of antigen)
Protocols
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Value-added Deliverables
① 200ug * antigen (positive control);
② 1ml * Pre-immune serum (negative control);
Quality Guarantee
① Antibody purity can be guaranteed above 90% by SDS-PAGE detection;
② ELISA titer can be guaranteed 1: 64,000;
③ WB validation with antigen can be guaranteed positive;
Lead Time
Made-to-order (14-16 weeks)

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Target Background

Function
Probable ion channel required for the normal function of cochlear hair cells.
Gene References into Functions
  1. We generated a model of TMC1 based on X-ray and cryo-EM structures of TMEM16 proteins, revealing the presence of a large cavity near the protein-lipid interface that also harbors the Beethoven mutation, suggesting that it could function as a permeation pathway. PMID: 30063209
  2. Transmembrane cochlear expressed gene 1 protein (TMC1)-dependent channels have larger single-channel conductance and in outer hair cells (OHCs) support a tonotopic apex-to-base conductance gradient. PMID: 29872055
  3. This study showed that calcium and integrin-binding protein 2 binds to the components of the hair cell mechanotransduction complex, TMC1 and TMC2, and these interactions are disrupted by deafness-causing Cib2 mutations. PMID: 28663585
  4. TMC1 and TMC2 are components of the stereocilia mechanoelectrical transduction channel complex. PMID: 26321635
  5. The results suggest that a major component of channel adaptation is regulated by changes in intracellular Ca(2+). PMID: 26324676
  6. This study demonstrated that the M412K point mutation in TMC1 of Beethoven mice leads to a reduced Ca2+ permeability, more so in Tmc1Bth/Bth than in Tmc1Bh/+, and conductance of the MET channel of Mouse Outer Hair Cells. PMID: 26758827
  7. gene augmentation with Tmc1 or Tmc2 is well suited for further development as a strategy for restoration of auditory function in deaf patients who carry TMC1 mutations PMID: 26157030
  8. During the first postnatal week, we observed a normal mechanotransducer current in hair cells lacking Tmc1 or Tmc2; however, in the absence of both isoforms, we recorded a large MT current that was phase-shifted 180 degrees . PMID: 24127526
  9. This study demonstrate TMC1 is components of hair cell transduction channels and contribute to permeation properties. PMID: 23871232
  10. Tmc1 is expressed in mouse vestibular & cochlear hair cells near the stereocilia tips. Deletion of Tmc1 & Tmc2 causes deafness. Restoration of Tmc1 rescues mechanotransduction. PMID: 22105175
  11. Tmc1 was present within the endoplasmic reticulum as an integral membrane protein containing six transmembrane domains and cytosolic N- and C-termini. PMID: 20672865
  12. role in mouse models of deafness [review] PMID: 14552423
  13. Excess of social and/or physical stimulation in Ts65Dn mice may affect cognition by disturbing the emotional and behavioral components of the learning process. PMID: 15941601

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Involvement in disease
Defects in Tmc1 are the cause of the dominant deaf mutant Beethoven (BTH). Heterozygotes show progressive hair-cell degeneration from day 20 onwards, leading to severe depletion of inner hair cells and scattered loss of outer hair cells, and progressive loss of the Preyer reflex from around day 30. Homozygotes show almost complete degeneration of inner hair cells, and little or no Preyer reflex at any age.; DISEASE: Note=Defects in Tmc1 are the cause of recessive deaf mutant dn. The dn mutant shows profound deafness with degeneration of the organ of Corti, stria vascularis, and occasionally the saccular macula, starting at about 10 days after birth (PubMed:11850618).
Subcellular Location
Cell membrane; Multi-pass membrane protein.
Protein Families
TMC family
Tissue Specificity
Detected in cochlear inner and outer hair cells and in neurosensory epithelia of the vestibular end organs. Also expressed in cortex, cerebellum, eye, colon, ovary and testis.
Database Links
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7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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