EXBIO Antibodies Product Data Sheet

1P-718-C025

Monoclonal Antibody to RLTPR / CARMIL2
Phycoerythrin (PE) conjugated (0.025 mg)

Clone: EM-53
Isotype: Mouse IgG1
Specificity: The mouse monoclonal antibody EM-53 recognizes RLTPR / CARMIL2, a protein playing a role in actin filament elongation.
Regulatory Status: RUO
Immunogen: Murine Rltpr (amino acids 1147-1397)
Species Reactivity: Human, Mouse
Preparation: The purified antibody is conjugated with R-Phycoerythrin (PE) under optimum conditions. The conjugate is purified by size-exclusion chromatography.
Concentration: 0.1 mg/ml
Storage Buffer: The reagent is provided in stabilizing phosphate buffered saline (PBS) solution containing 15mM sodium azide.
Storage / Stability: Store in the dark at 2-8°C. Do not freeze. Avoid prolonged exposure to light. Do not use after expiration date stamped on vial label.
Usage: The reagent is designed for Flow Cytometry analysis.
Expiration: See vial label
Lot Number: See vial label
Background: RLTPR / CARMIL2 (RGD motif, leucine rich repeats, tropomodulin domain and proline-rich containing; capping protein regulator and myosin 1 linker 2), also known as LRRC16C, is a cytosolic protein, which with high affinity binds CAPZA2 (capping protein muscle actin Z-line alpha 2) and decreases CAPZA2 affinity for actin barbed ends. RLTPR / CARMIL2 increases the rate of actin filament elongation from seeds in the presence of CAPZA2, however, seems unable to nucleate filaments. Its interaction with CAPZA2 is essential for lamellipodial protrusion and cell translocation. RLTPR / CARMIL2 is crutial for T cell costimulation via CD28 and this property seems to be independent on its actin-uncapping function. The lack of functional RLTPR / CARMIL2 molecules impeded the differentiation toward Th1 and Th17 fates of both human and murine CD4+ T cells and leads to combined immunodeficiency. Expression of RLTPR / CARMIL2 was also detected in human and murine B cells, but it seems not to be involved in BCR-mediated signaling.
References:
*Schober T, Magg T, Laschinger M, Rohlfs M, Linhares ND, Puchalka J, Weisser T, Fehlner K, Mautner J, Walz C, Hussein K, Jaeger G, Kammer B, Schmid I, Bahia M, Pena SD, Behrends U, Belohradsky BH, Klein C, Hauck F: A human immunodeficiency syndrome caused by mutations in CARMIL2. Nat Commun. 2017 Jan 23;8:14209.
*Liang Y, Cucchetti M, Roncagalli R, Yokosuka T, Malzac A, Bertosio E, Imbert J, Nijman IJ, Suchanek M, Saito T, Wülfing C, Malissen B, Malissen M: The lymphoid lineage-specific actin-uncapping protein Rltpr is essential for costimulation via CD28 and the development of regulatory T cells. Nat Immunol. 2013 Aug;14(8):858-66.
*Wang Y, Ma CS, Ling Y, Bousfiha A, Camcioglu Y, Jacquot S, Payne K, Crestani E, Roncagalli R, Belkadi A, Kerner G, Lorenzo L, Deswarte C, Chrabieh M, Patin E, Vincent QB, Müller-Fleckenstein I, Fleckenstein B, Ailal F, Quintana-Murci L, Fraitag S, Alyanakian MA, Leruez-Ville M, Picard C, Puel A, Bustamante J, Boisson-Dupuis S, Malissen M, Malissen B, Abel L, Hovnanian A, Notarangelo L, Jouanguy E, Tangye SG, Béziat V, Casanova JL: Dual T cell- and B cell-intrinsic deficiency in humans with biallelic RLTPR mutations. J Exp Med. 2016 Oct 17;213(11):2413-2435.
*Roncagalli R, Cucchetti M, Jarmuzynski N, Grégoire C, Bergot E, Audebert S, Baudelet E, Menoita MG, Joachim A, Durand S, Suchánek M, Fiore F, Zhang L, Liang Y, Camoin L, Malissen M4, Malissen B: The scaffolding function of the RLTPR protein explains its essential role for CD28 co-stimulation in mouse and human T cells. J Exp Med. 2016 Oct 17;213(11):2437-2457.
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