Key features and details
- Rabbit polyclonal to Nance-Horan Syndrome Protein
- Suitable for: ICC/IF, IHC-P
- Reacts with: Human
- Isotype: IgG
Product nameAnti-Nance-Horan Syndrome Protein antibody
DescriptionRabbit polyclonal to Nance-Horan Syndrome Protein
Tested applicationsSuitable for: ICC/IF, IHC-Pmore details
Species reactivityReacts with: Human
antigen sequence, corresponding to amino acids 1139-1281 of Human Nance-Horan Syndrome Protein.
- Human liver tissue.
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Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), 59% PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab122177 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use a concentration of 1 - 4 µg/ml.
Recommend PFA Fixation and Triton X-100 treatment
|IHC-P||1/20 - 1/50. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
FunctionUnknown. May have a key functions in the regulation of eye, tooth, brain and craniofacial development.
Tissue specificityDetected at low levels in all tissues analyzed. Detected in fetal and adult brain, lens, retina, retinal pigment epithelium, placenta, lymphocytes and fibroblasts. Levels in retinal pigment epithelium, placenta, lymphocytes, and fibroblasts are very low. Expressed also in kidney, lung and thymus.
Involvement in diseaseDefects in NHS are the cause of Nance-Horan syndrome (NHS) [MIM:302350]; also known as cataract-dental syndrome. NHS is a rare X-linked disorder characterized by congenital cataracts, dental anomalies, dysmorphic features, and, in some cases, mental retardation. Distinctive dental anomalies are seen in affected males, including supernumerary incisors and crown shaped permanent teeth. Characteristic facial features are anteverted pinnae, long face, and prominent nasal bridge and nose. Carrier females display milder variable symptoms of disease with lens opacities often involving the posterior Y sutures, and on occasion dental anomalies and the characteristic facial features described.
Defects in NHS are the cause of cataract congenital X-linked (CXN) [MIM:302200]. A X-linked form of cataract, manifesting as a congenital nuclear opacity with severe visual impairment in affected males. Heterozygous females have suture cataracts and only slight reduction in vision. Note=Caused by copy number variations predicted to result in altered transcriptional regulation of the NHS gene: a 0.8 Mb segmental duplication-triplication encompassing the NHS, SCML1 and RAI2 genes, and an 4.8 kb intragenic deletion in NHS intron 1.
Sequence similaritiesBelongs to the NHS family.
- Information by UniProt
- Congenital cataracts and dental anomalies protein antibody
- CXN antibody
- Nance-Horan syndrome protein antibody
Immunofluorescent staining of Human cell line U-2 OS shows positivity in nucleus but not nucleoli. Recommended concentration of ab122177 1-4 µg/ml. Cells treated with PFA/Triton X-100.
ab122177 at 1/35 dilution staining Nance-Horan Syndrome Protein in Paraffin-embedded Human Liver tissue by Immunohistochemistry.
ab122177 has not yet been referenced specifically in any publications.