SH2D1A Human

SH2 domain containing 1A Human Recombinant
Cat. No.
BT25376
Source
Escherichia Coli.
Synonyms
SH2 domain-containing protein 1A, Duncan disease SH2-protein, Signaling lymphocytic activation molecule-associated protein, SLAM-associated protein, T-cell signal transduction molecule SAP, SH2D1A, DSHP, SAP, LYP, XLP, EBVS, IMD5, XLPD, MTCP1, FLJ18687, FLJ92177, SAP/SH2D1A.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 95.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

SH2D1A Human Recombinant fused with a 20 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 148 amino acids (1-128 a.a.) and having a molecular mass of 16.3kDa. The SH2D1A is purified by proprietary chromatographic techniques.

Product Specs

Introduction
SH2D1A, also known as Signaling Lymphocytic Activation Molecule (SLAM)-associated protein, functions as an inhibitor of SLAM self-association. Highly expressed in the thymus and lungs, with lower expression in the spleen and liver, SH2D1A plays a critical role in the bidirectional stimulation of T and B cells. Structurally, it comprises an SH2 domain and a short tail. Defects in the SH2D1A gene are linked to X-linked lymphoproliferative disease (XLPD), also known as Duncan disease, a rare congenital immunodeficiency characterized by severe complications following Epstein-Barr virus (EBV) infection.
Description
This product consists of the human recombinant SH2D1A protein, inclusive of a 20 amino acid His tag positioned at the N-terminus. Expressed in E. coli, it presents as a single, non-glycosylated polypeptide chain, encompassing 148 amino acids (specifically, amino acids 1 to 128). With a molecular weight of 16.3 kDa, the SH2D1A protein undergoes purification through proprietary chromatographic methods.
Physical Appearance
Clear, colorless solution that has been sterilized by filtration.
Formulation
The SH2D1A protein is supplied in a solution at a concentration of 1 mg/ml. The solution is buffered with 20mM Tris-HCl at a pH of 7.5 and further contains 1mM DTT and 10% glycerol.
Stability
For short-term storage (2-4 weeks), the product should be kept refrigerated at 4°C. For extended storage, it is recommended to freeze the product at -20°C. To ensure optimal stability during long-term storage, consider adding a carrier protein (either HSA or BSA) to a final concentration of 0.1%. Repeated freezing and thawing of the product should be avoided.
Purity
The purity of the SH2D1A protein is greater than 95.0% as determined by SDS-PAGE analysis.
Synonyms
SH2 domain-containing protein 1A, Duncan disease SH2-protein, Signaling lymphocytic activation molecule-associated protein, SLAM-associated protein, T-cell signal transduction molecule SAP, SH2D1A, DSHP, SAP, LYP, XLP, EBVS, IMD5, XLPD, MTCP1, FLJ18687, FLJ92177, SAP/SH2D1A.
Source
Escherichia Coli.
Amino Acid Sequence

MGSSHHHHHH SSGLVPRGSH MDAVAVYHGK ISRETGEKLL LATGLDGSYL LRDSESVPGV YCLCVLYHGY IYTYRVSQTE TGSWSAETAP GVHKRYFRKI KNLISAFQKP DQGIVIPLQY PVEKKSSARS TQGTTGIRED PDVCLKAP.

Product Science Overview

Introduction

SH2 domain containing 1A (SH2D1A), also known as Signaling Lymphocytic Activation Molecule-Associated Protein (SLAM-associated protein or SAP), is a protein encoded by the SH2D1A gene in humans. This protein plays a crucial role in the immune system, particularly in the signaling pathways of T and B cells .

Structure and Function

The SH2D1A protein contains an SH2 (Src Homology 2) domain, which is a structurally conserved protein domain found in many intracellular signal-transducing proteins . The SH2 domain allows the protein to bind to phosphorylated tyrosine residues on other proteins, thereby modifying the function or activity of the SH2-containing protein . This binding is essential for the transmission of signals that control various cellular functions.

Role in Immune System

SH2D1A is primarily involved in the bidirectional stimulation of T and B cells. It associates with the signaling lymphocyte-activation molecule (SLAM), acting as an inhibitor by blocking the recruitment of the SH2-domain-containing signal-transduction molecule SHP-2 to its docking site . Additionally, SH2D1A can bind to other related surface molecules expressed on activated T, B, and NK cells, thereby modifying signal transduction pathways in these cells .

Clinical Significance

Mutations in the SH2D1A gene are associated with lymphoproliferative syndrome X-linked type 1 (XLP1), also known as Duncan disease . This rare immunodeficiency disorder is characterized by extreme susceptibility to infection with the Epstein-Barr virus (EBV), leading to severe mononucleosis and malignant lymphoma . Multiple transcript variants encoding different isoforms of SH2D1A have been identified, highlighting the complexity of its regulation and function .

Research and Applications

The recombinant form of SH2D1A is used in various research applications to study its role in immune signaling pathways and its involvement in diseases. Understanding the function and regulation of SH2D1A can provide insights into potential therapeutic targets for treating immune-related disorders.

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