Anti-TRPS1 Antibody Picoband® fluoro488 Vervoeging

Zinc finger transcription factor Trps1 is a protein that in humans is encoded by the TRPS1 gene. This gene encodes a transcription factor that represses GATA-regulated genes and binds to a dynein light chain protein. Binding of the encoded protein to the dynein light chain protein affects binding to GATA consensus sequences and suppresses its transcriptional activity. Defects in this gene are a cause of tricho-rhino-phalangeal syndrome (TRPS) types I-III.

Mannan-binding lectin serine protease 2; MBL-associated serine protease 2; Mannose-binding protein-associated serine protease 2; MASP-2; MASP2

TRPS1

7227

Q9UHF7

Human

No cross-reactivity with other proteins.

E.coli-derived human TRPS1 recombinant protein (Position: H353-D1257) .

Plasma.

Flow Cytometry

Signal Transduction

Immunogen affinity purified.

Liquid

Serum protease that plays an important role in the activation of the complement system via mannose-binding lectin. After activation by auto-catalytic cleavage it cleaves C2 and C4, leading to their activation and to the formation of C3 convertase.

1. Balducci, R., Toscano, V., Tedeschi, B., Mangiantini, A., Toscano, R., Galasso, C., Cianfarani, S., Boscherini, B. A new case of Ambras syndrome associated with a paracentric inversion (8) (q12; q22) . Clin. Genet. 53: 466-468, 1998. 2. Baumeister, F. A. M., Egger, J., Schildhauer, M. T., Stengel-Rutkowski, S. Ambras syndrome: delineation of a unique hypertrichosis universalis congenita and association with a balanced pericentric inversion (8) (p11.2; q22) . Clin. Genet. 44: 121-128, 1993. 3. Fantauzzo, K. A., Kurban, M., Levy, B., Christiano, A. M. Trps1 and its target gene Sox9 regulate epithelial proliferation in the developing hair follicle and are associated with hypertrichosis. PLoS Genet. 8: e1003002, 2012. Note: Electronic Article.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

33927 MW

6

Mannan binding lectin serine peptidase 2

Secreted.

Mannan-binding lectin serine protease 2

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.