Code
OSS00287W
$240USD
ID Tag
Rb1530-130810-WS
Immunogen
A synthetic peptide from c-terminal region of human Pendrin (SLC26A4) conjugated to an immunogenic carrier protein was used as the antigen.
Accession
Also known
Sodium-independent chloride/iodide transporter, Solute carrier family 26 member 4, SLC26A4, PDS
Target
Function: Sodium-independent transporter of chloride and iodide.
Defects in SLC26A4 are a cause of Pendred syndrome (PDS)
. PDS is an autosomal recessive disorder characterized by congenital sensorineural hearing loss combined with thyroid goiter. The disorder may account for up to 10% of the cases of hereditary deafness. The deafness is most often associated with a Mondini cochlear defect.
Defects in SLC26A4 are the cause of deafness autosomal recessive type 4 (DFNB4)
; also known as vestibular aqueduct syndrome (EVA). DFNB4 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. DFNB4 is associated with an enlarged vestibular aqueduct.
Tissue specificity: High expression in adult thyroid, lower expression in adult and fetal kidney and fetal brain. Not expressed in other tissues.
SUBCELLULAR LOCATION: Membrane; Multi-pass membrane protein
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
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Everett L.A, et al. Nat. Genet. 17:411-422(1997)
2. Scott D.A, et al. Nat. Genet. 21:440-443(1999)
3. Coyle B, et al. Hum. Mol. Genet. 7:1105-1112(1998)
Limitation
For research use only
Code
OSS00287W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC, WB (confirmed by recombinant protein). A dilution of 1: 200 to 1: 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for SLC26A4.
Spcs X-react.
Human. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.