Anti-RLTPR Purified

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.

Product Specifications

Certification

RUO

Reactivity

Mouse, Human

Immunogen

Murine RLTPR

Target Antigen

RLTPR

Clone

EM-53

Applications

FC (QC tested), WB

Concentration

1 mg/mL

Format

Purified

Buffer

Phosphate buffered saline (PBS), pH 7.4, 15 mM sodium azide

References & Citations

*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., URL: https://pubmed.ncbi.nlm.nih.gov/27647349/, *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., URL: https://www.ncbi.nlm.nih.gov/pubmed/27647348 , *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., URL: https://www.ncbi.nlm.nih.gov/pubmed/23793062

Other 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., URL: https://www.ncbi.nlm.nih.gov/pubmed/28112205