Rb3149-241117-WS

A synthetic peptide from human TMC1 conjugated to blue carrier protein was used as the antigen. The peptide is homologous in mouse.

TMC1_HUMAN
TMC1_MOUSE

Transmembrane channel-like protein 1, transmembrane cochlear-expressed protein 1, Beethoven protein, Deafness protein, CWEA1, Bth, dn

FUNCTION: Required for the normal function of cochlear hair cells.
SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein.
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.
DEVELOPMENTAL STAGE: Expressed at low, constant levels in temporal bone from embryonic day 14 to day 1 after birth. Increases by 8 to 16-fold at day 5, 10 and 20 and continues to be expressed up to day 90.
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: 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.

Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

12 months after reconstitution

This item will be shipped to you at ambient temperature in a lyophilised form.

1. Vreugde S, et al. Nat. Genet. 30:257-258(2002).

For research use only

100 µl

Unconjugated antibody

NZ white rabbit

Whole serum

Polyclonal

Polyclonal, whole serum

IHC, WB. A dilution of 1 : 1000 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Specific for TMC1.

Human, mouse. Other species not yet tested.

Lyophilised

Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.

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