MOUSE ANTI SOS1

Manufacturer GENWAY
Category
Type Antibody
Specific against other
Applications WB, IF
Amount 0.1 mg
Host Mouse
ArtNr 20-783-314760
Available
Genway ID:
GWB-A601C4
Specificity:
SOS1
Isotype:
IgG1Species Cross Reactivity: Reacts with: MouseN. B. Antibody reactivity and working conditions may vary between species.
Preparation:
Purified IgG prepared by affinity chromatography on Protein A
Buffer Solution:
Phosphate buffered saline pH7. 4
Preservative Stabilisers:
0. 09% Sodium Azide (NaN3)Approx. Protein Concentrations: IgG concentration 1. 0mg/ml
Immunogen:
Synthetic peptide corresponding to amino acids THPSMHRDGPPLLENAHSS of human SOS1.
Specificity:
Specifically recognises SOS1 (son of sevenless homolog 1) a ubiquitously expressed 150kDa intracellular protein and bifunctional guanine nucleotide exchange factor (GEF) which plays a role in growth and differentiation signalling pathways and in actin reorganization by acting as a regulator of Ras and Rac/Rho GTPases. Mutations in the SOS1 gene are responsible for the rare oral overgrowth condition known as gingival fibromatosis 1 (GINGF1) and also the cause of the relatively common autosomal dominant disorder Noonan syndrome type 4 (NS4) characterized by short stature congenital heart malformation bleeding diathesis and distinctive facial features. Recommended Secondary Antibodies: Rabbit Anti Mouse IgGGoat Anti Mouse IgGGoat Anti Mouse IgG (H/L)Goat Anti Mouse IgG IgA IgMHuCAL Anti Mouse IgG1Goat Anti Mouse IgG (Fc)Sheep Anti Mouse IgG (H/L)
Function:
Promotes the exchange of Ras-bound GDP by GTP. Subunit structureInteracts with GRB2. Forms a complex with phosphorylated MUC1 and GRB2 (via its SH3 domains). Interacts with phosphorylated LAT2. Ref. 4Ref. 5Tissue specificityExpressed in gingival tissues. Ref. 11Involvement in diseaseDefects in SOS1 are the cause of gingival fibromatosis 1 (GGF1) [MIM:135300]; also known as GINGF1. Gingival fibromatosis is a rare overgrowth condition characterized by a benign slowly progressive nonhemorrhagic fibrous enlargement of maxillary and mandibular keratinized gingiva. GGF1 is usually transmitted as an autosomal dominant trait although sporadic cases are common. Ref. 11Defects in SOS1 are the cause of Noonan syndrome type 4 (NS4) [MIM:610733]. NS4 is an autosomal dominant disorder characterized by dysmorphic facial features short stature hypertelorism cardiac anomalies deafness motor delay and a bleeding diathesis. It is a genetically heterogeneous and relatively common syndrome with an estimated incidence of 1 in 1000-2500 live births. Rarely NS4 is associated with juvenile myelomonocytic leukemia (JMML). SOS1 mutations engender a high prevalence of pulmonary valve disease; atrial septal defects are less common. Ref. 12Ref. 13Sequence similaritiesContains 1 DH (DBL-homology) domain. Contains 1 N-terminal Ras-GEF domain. Contains 1 PH domain. Contains 1 Ras-GEF domain.
Amount: 0.1 mg
Available: In stock
Listprice: €389.48
Discount: -10.0%
Price: €350.53
You save: €38.95
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