Anti-GNAS antibody produced in rabbit,100UL,SAB2100941-100UL,Sigma

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订货号 0BP0373
品牌型号 Sigma SAB2100941-100UL
货期 询货期
最小订货量 1件
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产品介绍 Product Description

Immunogen

Synthetic peptide directed towards the N terminal region of human GNAS


Biochem/physiol Actions

Mutations in GNAS gene result in pseudohypoparathyroidism type 1a, pseudohypoparathyroidism type 1b, Albright hereditary osteodystrophy, pseudopseudohypoparathyroidism, McCune-Albright syndrome, progressive osseus heteroplasia, polyostotic fibrous dysplasia of bone, and some pituitary tumors. This locus has a highly complex imprinted expression pattern. It gives rise to maternally, paternally, and biallelically expressed transcripts that are derived from four alternative promoters and 5′ exons. Some transcripts contains a differentially methylated region (DMR) at their 5′ exons, and this DMR is commonly found in imprinted genes and correlates with transcript expression. An antisense transcript exists, and this antisense transcript and one of the transcripts are paternally expressed, produce noncoding RNAs, and may regulate imprinting in this region. In addition, one of the transcripts contains a second overlapping ORF, which encodes a structurally unrelated protein - Alex. Alternative splicing of downstream exons is also observed, which results in different forms of the stimulatory G-protein alpha subunit, a key element of the classical signal transduction pathway linking receptor-ligand interactions with the activation of adenylyl cyclase and a variety of cellular reponses. Multiple transcript variants have been found for this gene, but the full-length nature and/or biological validity of some variants have not been determined. Mutations in this gene result in pseudohypoparathyroidism type 1a, pseudohypoparathyroidism type 1b, Albright hereditary osteodystrophy, pseudopseudohypoparathyroidism, McCune-Albright syndrome, progressive osseus heteroplasia, polyostotic fibrous dysplasia of bone, and some pituitary tumors.


Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.


Other Notes

Synthetic peptide located within the following region: VYRATHRLLLLGAGESGKSTIVKQMRILHVNGFNGEGGEEDPQAARSNSD


Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.


技术参数 Specifications
biological sourcerabbit
Quality Level100
conjugateunconjugated
antibody formaffinity isolated antibody
antibody product typeprimary antibodies
clonepolyclonal
formbuffered aqueous solution
mol wt 46 kDa
species reactivityguinea pig, horse, dog, bovine, rat, mouse, human
concentration0.5 mg - 1 mg/mL
technique(s)immunohistochemistry: suitable,western blot: suitable
UniProt accession no.P63092
shipped inwet ice
storage temp.−20°C
target post-translational modificationunmodified
Gene Informationhuman ... GNAS(2778)
长度(mm)
宽度(mm)
高度(mm)
重量(kg)
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