Product Overview
Description
CLPP-00151017 is recombinant human PSTPIP1 protein
Protein Length
Full length protein
Nature
Recombinant Protein
Sequence
MGSSHHHHHHSSGLVPRGSHMGSMMPQLQFKDAFWCRDFTAHTGYEVLLQRLLDGRKMCKDMEELLRQRAQAEERYGKELVQIARKAGGQTEINSLRASFDSLKQQMENVGSSHIQLALTLREELRSLEEFRERQKEQRKKYEAVMDRVQKSKLSLYKKAMESKKTYEQKCRDADDAEQAFERISANGHQKQVEKSQNKARQCKDSATEAERVYRQSIAQLEKVRAEWEQEHRTTCEAFQLQEFDRLTILRNALWVHSNQLSMQCVKDDELYEEVRLTLEGCSIDADIDSFIQAKSTGTEPPAPVPYQNYYDREVTPLTSSPGIQPSCGMIKRFSGLLHGSPKTTSLAASAASTETLTPTPERNEGVYTAIAVQEIQGNPASPAQEYRALYDYTAQNPDELDLSAGDILEVILEGEDGWWTVERNGQRGFVPGSYLEKL
Sequence Similarities
Contains 1 FCH domain. Contains 1 SH3 domain.
Predicted Molecular Weight
50 kDa including tags
Target Information
Alternative Names
CD2 antigen binding protein 1; CD2 binding protein 1; CD2 cytoplasmic tail binding protein; CD2-binding protein 1; CD2BP1; CD2BP1L; CD2BP1S; H PIP; H-PIP; HPIP; PAPAS; PEST phosphatase interacting protein 1; PEST phosphatase-interacting protein 1; PPIP1_HUMAN; Proline serine threonine phosphatase interacting protein 1; Proline-serine-threonine phosphatase-interacting protein 1; PSTPIP; Pstpip1
Protein Function
Involved in regulation of the actin cytoskeleton. May regulate the WAS actin-bundling activity. Bridges the interaction between ABL1 and PTPN18 leading to the ABL1 dephosphorylation. May play a role as a scaffold protein between PTPN12 and WAS and allows PTPN12 to dephosphorylate WAS. Has the potential to physically couple CD2 and CD2AP to WAS. Acts downstream of CD2 and CD2AP to recruit WAS to the T-cell:APC contact site so as to promote the actin polymerization required for synapse induction during T-cell activation (By similarity). Down-regulates CD2-stimulated adhesion through the coupling of PTPN12 to CD2.
Tissue Specificity
Highly expressed in the peripheral blood leukocytes, granulocytes and monocytes, namely in T-cells and natural killer cells, and in spleen. Weakly expressed in the thymus, small intestine, lung and placenta.
Involvement in Disease
Defects in PSTPIP1 are the cause of PAPA syndrome (PAPAS); also known as pyogenic sterile arthritis, pyoderma gangrenosum and acne or familial recurrent arthritis (FRA). PAPAS is characterized by autosomal dominant inheritance of early onset, primarily affecting skin and joint tissues. Recurring inflammatory episodes lead to accumulation of sterile, pyogenic, neutrophil-rich material within the affected joints, ultimately resulting in significant destruction.
Shipping & Handling
Constituents
0.02% DTT, 0.32% Tris HCl, 20% Glycerol (glycerin, glycerine), 0.88% Sodium chloride.
Shipping
Shipped at 4 °C.
Storage
Store at -20 °C or -80 °C.
