TMC1 antibody, Beethoven antibody, deafness protein antibody

Rabbit antibody to 4th cytoplasmic loop of human, mouse TMC1 (Beethoven/deafness protein, CWEA1, Bth, dn): whole serum

$240USD

Code	OST00173W
ID Tag	Rb552-100808-WS
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Unit size	100 µl
Immunogen	A synthetic peptide from the 4th cytoplasmic loop of human transmembrane channel-like protein 1 (TMC1, Beethoven/deafness protein, CWEA1, Bth, dn) conjugated to an immunogenic carrier protein was used as the immunogen. The antigen is homologous with the corresponding sequence in mouse.
Conjugate	Unconjugated antibody
Accession	TMC1_HUMAN TMC1_MOUSE
Also known as	Transmembrane channel-like protein 1, transmembrane cochlear-expressed protein 1, Beethoven protein, Deafness protein, CWEA1, Bth, dn, 4933416G09Rik
Background	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.
Host	NZ white rabbit
Purity	Whole serum
Clonality	Polyclonal
Isotype	Polyclonal, whole serum
Applications	IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity	Specific for TMC1.
Spcs X-react.	Human, mouse. Other species not yet tested.
Format	Lyophilised
Reconstitution	Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.
Storage	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.
Expiry Date	12 months after reconstitution
Transportation	This item will be shipped to you at ambient temperature in a lyophilised form.
References	1. Vreugde S, et al. Nat. Genet. 30:257-258(2002). 2. Keresztes G, et al. BMC Genomics 4:24-24(2003). 3. Kurima K, et al. Genomics 82:300-308(2003). 4. Carninci P, et al. Science 309:1559-1563(2005). 5. Kurima K, et al. Nat. Genet. 30:277-284(2002). 6. Humphray S.J, et al. Nature 429:369-374(2004). 7. Ota T, et al. Nat. Genet. 36:40-45(2004).
Limitation	For research use only

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