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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