Code
OSS00306W
$240USD
ID Tag
Rb1718-020111-WS
Immunogen
A synthetic peptide to the internal region of human SHOX conjugated to an immunogenic carrier protein was used as the antigen.
Accession
Also known
PHOG, Short stature homeobox protein, Pseudoautosomal homeobox-containing osteogenic protein, Short stature homeobox-containing protein
Target
FUNCTION: Controls fundamental aspects of growth and development.
Subcellular location: Nucleus
Tissue specificity: SHOXA is expressed in skeletal muscle, placenta, pancreas, heart and bone marrow fibroblast and SHOXB is highly expressed in bone marrow fibroblast followed by kidney and skeletal muscle. SHOXB is not expressed in brain, kidney, liver and lung. Highly expressed in osteogenic cells.
Defects in SHOX are the cause of Leri-Weill dyschondrosteosis (LWD)
. LWD is a dominantly inherited skeletal dysplasia characterized by moderate short stature predominantly because of short mesomelic limb segments. It is often associated with the Madelung deformity of the wrist, comprising bowing of the radius and dorsal dislocation of the distal ulna.
Defects in SHOX are a cause of Langer mesomelic dysplasia (LMD)
. LMD is an autosomal recessive rare skeletal dysplasia characterized by severe short stature owing to shortening and maldevelopment of the mesomelic and rhizomelic segments of the limbs. Associated malformations are rarely reported and intellect is normal in all affected subjects reported to date.
Defects in SHOX are a cause of idiopathic short stature (ISS)
. Idiopathic short stature is usually defined as a height below the third percentile for chronological age or minus 2 standard deviations of national height standards in the absence of specific causative disorders.
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. Rao E, et al. Nat. Genet. 16:54-63(1997)
2. Ellison J.W, et al. Hum. Mol. Genet. 6:1341-1347(1997)
3. Huber C, et al. J. Med. Genet. 38:323-323(2001)
Limitation
For research use only
Code
OSS00306W
$240USD
Unit size
100 µl
Conjugate
Unconjugated antibody
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 SHOX.
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.