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Anti-TRPS1 Antibody Picoband® Fluoro488 Conjugated

Product Specifications

Background

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.

Synonyms

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

Gene Name

TRPS1

Gene ID

7227

UniProt

Q9UHF7

Host

Rabbit

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

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

Clonality

Polyclonal

Tissue Specificity

Plasma.

Applications

Flow Cytometry

Field of Research

Signal Transduction

Purification

Immunogen affinity purified.

Form

Liquid

Function

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.

References & Citations

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.

Storage Conditions

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

Calculated Molecular Weight

33927 MW

Applications Notes

6

Gene Name Synonym

Mannan binding lectin serine peptidase 2

Subcellular Location

Secreted.

Protein Name

Mannan-binding lectin serine protease 2

Isotype

Rabbit IgG

Contents

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

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