Anti-Utrophin/UTRN Antibody Picoband® Fluoro488 Conjugated

Utrophin is a protein that in humans is encoded by the UTRN gene. This gene shares both structural and functional similarities with the dystrophin gene. It contains an actin-binding N-terminus, a triple coiled-coil repeat central region, and a C-terminus that consists of protein-protein interaction motifs which interact with dystroglycan protein components. The protein encoded by this gene is located at the neuromuscular synapse and myotendinous junctions, where it participates in post-synaptic membrane maintenance and acetylcholine receptor clustering. Mouse studies suggest that this gene may serve as a functional substitute for the dystrophin gene and therefore, may serve as a potential therapeutic alternative to muscular dystrophy which is caused by mutations in the dystrophin gene. Alternative splicing of the utrophin gene has been described; however, the full-length nature of these variants has not yet been determined.

Almost ubiquitous. Largely expressed in adult heart, skeletal muscle, ovary, and fetal kidney. Moderate amounts in adult liver, kidney, pancreas, spleen, and fetal lung, and small amounts in placenta, adult lung, prostate, testis, colon, fetal brain and liver.

Cancer, Developmental Families, Domain Families, Epigenetics and Nuclear Signaling, G Protein Signaling, Oncoproteins/Suppressors, Signal Transduction, Signaling Pathway, Signaling Pathways, Stem Cells, Surface Molecules, Transcription, Tumor Suppressors

Receptor for Wnt proteins. Most of frizzled receptors are coupled to the beta-catenin (CTNNB1) canonical signaling pathway, which leads to the activation of disheveled proteins, inhibition of GSK-3 kinase, nuclear accumulation of beta-catenin (CTNNB1) and activation of Wnt target genes. Plays a critical role in retinal vascularization by acting as a receptor for Wnt proteins and norrin (NDP) . In retina, it can be both activated by Wnt protein-binding, but also by a Wnt-independent signaling via binding of norrin (NDP), promoting in both cases beta-catenin (CTNNB1) accumulation and stimulation of LEF/TCF-mediated transcriptional programs. A second signaling pathway involving PKC and calcium fluxes has been seen for some family members, but it is not yet clear if it represents a distinct pathway or if it can be integrated in the canonical pathway, as PKC seems to be required for Wnt-mediated inactivation of GSK-3 kinase. Both pathways seem to involve interactions with G-proteins. May be involved in transduction and intercellular transmission of polarity information during tissue morphogenesis and/or in differentiated tissues.

1. Blake, D. J., Love, D. R., Tinsley, J., Morris, G. E., Turley, H., Gatter, K., Dickson, G., Edwards, Y. H., Davies, K. E. Characterization of a 4.8kb transcript from the Duchenne muscular dystrophy locus expressed in schwannoma cells. Hum. Molec. Genet. 1: 103-109, 1992. 2. Blake, D. J., Schofield, J. N., Zuellig, R. A., Gorecki, D. C., Phelps, S. R., Barnard, E. A., Edwards, Y. H., Davies, K. E. G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain. Proc. Nat. Acad. Sci. 92: 3697-3701, 1995. 3. Buckle, V. J., Guenet, J. L., Simon-Chazottes, D., Love, D. R., Davies, K. E. Localisation of a dystrophin-related autosomal gene to 6q24 in man, and to mouse chromosome 10 in the region of the dystrophia muscularis (dy) locus. Hum. Genet. 85: 324-326, 1990.