Overview

  • Product name

    Anti-GNAS antibody
  • Description

    Rabbit Polyclonal to GNAS
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human G protein alpha S aa 920-970 conjugated to Keyhole Limpet Haemocyanin (KLH). The exact sequence is proprietary. from isoform Isoform XLas-1.
    Sequence:

    W NNRWLRTISV ILFLNKQDLL AEKVLAGKSK IEDYFPEFAR YTTPEDATPE


    Database link: Q5JWF2-1

  • Positive control

    • Human lung carcinoma and rat brain tissues.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    Preservative: 0.09% Sodium azide
    Constituents: 50% Glycerol, 0.01% BSA
  • Concentration information loading...
  • Purity

    Protein A purified
  • Clonality

    Polyclonal
  • Isotype

    IgG

Applications

Our Abpromise guarantee covers the use of ab203194 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/100 - 1/1000. Predicted molecular weight: 111 kDa.
IHC-P 1/100 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

Use at 1/50 - 1/200 with fluorescent detection methods.

Target

  • Function

    Guanine nucleotide-binding proteins (G proteins) are involved as modulators or transducers in various transmembrane signaling systems. The G(s) protein is involved in hormonal regulation of adenylate cyclase: it activates the cyclase in response to beta-adrenergic stimuli.
  • Involvement in disease

    Albright hereditary osteodystrophy (AHO) [MIM:103580]: A disorder characterized by short stature, obesity, round facies, brachydactyly and subcutaneous calcification. It is often associated with pseudohypoparathyoidism, hypocalcemia and elevated PTH levels. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Pseudohypoparathyroidism 1A (PHP1A) [MIM:103580]: A disorder characterized by end-organ resistance to parathyroid hormone, hypocalcemia and hyperphosphatemia. It is commonly associated with Albright hereditary osteodystrophy whose features are short stature, obesity, round facies, short metacarpals and ectopic calcification. Note=The disease is caused by mutations affecting the gene represented in this entry.
    McCune-Albright syndrome (MAS) [MIM:174800]: Characterized by polyostotic fibrous dysplasia, cafe-au-lait lesions, and a variety of endocrine disorders, including precocious puberty, hyperthyroidism, hypercortisolism, growth hormone excess, and hyperprolactinemia. The mutations producing MAS lead to constitutive activation of GS alpha. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Growth hormone-secreting pituitary adenoma (GHSPA) [MIM:102200]: Pituitary adenomas include somatotropinoma and prolactinoma. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Progressive osseous heteroplasia (POH) [MIM:166350]: Rare autosomal dominant disorder characterized by extensive dermal ossification during childhood, followed by disabling and widespread heterotopic ossification of skeletal muscle and deep connective tissue. Note=The disease is caused by mutations affecting the gene represented in this entry.
    ACTH-independent macronodular adrenal hyperplasia (AIMAH) [MIM:219080]: A rare adrenal defect characterized by multiple, bilateral, non-pigmented, benign, adrenocortical nodules. It results in excessive production of cortisol leading to ACTH-independent Cushing syndrome. Clinical manifestations of Cushing syndrome include facial and trunkal obesity, abdominal striae, muscular weakness, osteoporosis, arterial hypertension, diabetes. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Pseudohypoparathyroidism 1B (PHP1B) [MIM:603233]: A disorder characterized by end-organ resistance to parathyroid hormone, hypocalcemia and hyperphosphatemia. Patients affected with PHP1B lack developmental defects characteristic of Albright hereditary osteodystrophy, and typically show no other endocrine abnormalities besides resistance to PTH. Note=The disease is caused by mutations affecting the gene represented in this entry. Most affected individuals have defects in methylation of the gene. In some cases microdeletions involving the STX16 appear to cause loss of methylation at exon A/B of GNAS, resulting in PHP1B. Paternal uniparental isodisomy have also been observed.
    GNAS hyperfunction (GNASHYP) [MIM:139320]: This condition is characterized by increased trauma-related bleeding tendency, prolonged bleeding time, brachydactyly and mental retardation. Both the XLas isoforms and the ALEX protein are mutated which strongly reduces the interaction between them and this may allow unimpeded activation of the XLas isoforms. Note=The disease is caused by mutations affecting the gene represented in this entry.
    Pseudohypoparathyroidism 1C (PHP1C) [MIM:612462]: A disorder characterized by end-organ resistance to parathyroid hormone, hypocalcemia and hyperphosphatemia. It is commonly associated with Albright hereditary osteodystrophy whose features are short stature, obesity, round facies, short metacarpals and ectopic calcification. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • Sequence similarities

    Belongs to the G-alpha family. G(s) subfamily.
  • Cellular localization

    Cell membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • Adenylate cyclase-stimulating G alpha protein antibody
    • AHO antibody
    • alternative gene product encoded by XL-exon antibody
    • C20orf45 antibody
    • extra large alphas protein antibody
    • GNAS antibody
    • GNAS complex locus antibody
    • GNAS1 antibody
    • GNAS1 gene, formerly antibody
    • GNAS1, formerly antibody
    • GNAS2_HUMAN antibody
    • GPSA antibody
    • GSA antibody
    • GSP antibody
    • guanine nucleotide binding protein (G protein), alpha stimulating activity polypeptide 1 antibody
    • guanine nucleotide regulatory protein antibody
    • Guanine nucleotide-binding protein G(s) subunit alpha isoforms short antibody
    • guanine nucleotide-binding protein G(s) subunit alpha isoforms XLas antibody
    • NESP antibody
    • neuroendocrine secretory protein antibody
    • PHP1A antibody
    • PHP1B antibody
    • PHP1C antibody
    • POH antibody
    • protein ALEX antibody
    • secretogranin VI antibody
    see all

Images

  • Immunohistochemical analysis of formalin-fixed paraffin-embedded rat brain tissue, labeling G protein alpha S using ab203194 at a 1/200 dilution, followed by conjugation to the secondary antibody and DAB staining.

  • Immunohistochemical analysis of formalin-fixed paraffin-embedded Human lung carcinoma tissue, labeling G protein alpha S using ab203194 at a 1/200 dilution, followed by conjugation to the secondary antibody and DAB staining.

References

This product has been referenced in:

  • Taneera J  et al. GNAS gene is an important regulator of insulin secretory capacity in pancreatic ß-cells. Gene 715:144028 (2019). Read more (PubMed: 31374326) »
See 1 Publication for this product

Customer reviews and Q&As

Application
Immunohistochemistry (Frozen sections)
Sample
Mouse Tissue sections (Heart)
Permeabilization
Yes - 0.3% Triton X-100
Specification
Heart
Blocking step
BSA as blocking agent for 2 hour(s) and 0 minute(s) · Concentration: 1% · Temperature: RT°C
Fixative
Paraformaldehyde

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Verified customer

Submitted May 13 2016

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