catalog number :
MBS6005634
products full name :
Utrophin, NT (UTRN, DMDL, Dystrophin-related Protein 1, DRP1, FLJ23678, DRP)
products short name :
Utrophin
products name syn :
Anti -Utrophin, NT (UTRN, DMDL, Dystrophin-related Protein 1, DRP1, FLJ23678, DRP)
other names :
utrophin; Utrophin; utrophin; DRP-1; dystrophin-related protein 1; utrophin; Dystrophin-related protein 1
other gene names :
UTRN; UTRN; DRP; DMDL; DRP1; DMDL; DRP1; DRP-1
uniprot entry name :
UTRO_HUMAN
reactivity :
Canine, Human, Rat
specificity :
Recognizes Utrophin. Species Crossreactivity: Canine, human and rat.
form :
Supplied as a lyophilized powder in 15mM sodium azide. Reconstitute with 2.5ml dH2O.
storage stability :
Lyophilized powder may be stored at 4 degree C. Stable for 12 months after receipt at 4 degree C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
tested application :
Immunohistochemistry (IHC)
app notes :
Suitable for use in Immunohistochemistry and Electron Microscopy Gold. Not suitable for Western Blot or Paraffin. Other applications not tested. Dilution: Immunohistochemistry: 1:2-1:10 for 1 hr. at RT. Electron Microscopy Gold: Neat. Light fixation with 2% formaldehyde and 0.001% glutaraldehyde for 1 hour, 2.3M sucrose used as cryoprotectant is recommended for 90 min. at 25 degree C. Optimal dilutions to be determined by the researcher
other info1 :
Immunogen: Fusion protein consisting of the first 261aa of the published DMDL gene sequence. Grade: Supernatant
other info2 :
Positive Control: Immunohistochemistry: In normal adult human muscle the immunostaining is limited to neuromuscular junctions (NMJs). Electron Microscopy Gold: Staining pattern atnmJs.
products categories :
Antibodies; Abs to Neuroscience
products description :
Utrophin is a homologue of dystrophin and in normal muscle utrophin expression is restricted to neuromuscular junctions. Interestingly, in dystrophindeficient muscle, utrophin may be upregulated and is also present around the periphery of most muscle fibers. This antibody will help identify utrophin expression in muscle sections.
ncbi acc num :
NP_009055.2
ncbi gb acc num :
NM_007124.2
ncbi mol weight :
394,466 Da
ncbi summary :
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. [provided by RefSeq, Jul 2008]
uniprot summary :
Function: May play a role in anchoring the cytoskeleton to the plasma membrane . By similarity. Subunit structure: Interacts with the syntrophins SNTA1; SNTB1 and SNTB2. Interacts with SYNM. Interacts (via its WWW and ZZ domains) with DAG1 (via the PPXY motif of betaDAG1); the interaction is inhibited by the tyrosine phosphorylation of the PPXY motif of DAG1. Ref.4 Ref.5 Ref.6 Ref.7 Ref.8. Subcellular location: Cell junction synapse postsynaptic cell membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm cytoskeleton. Note: Neuromuscular junction. Tissue specificity: Muscle. Sequence similarities: Contains 2 CH (calponin-homology) domains.Contains 20 spectrin repeats.Contains 1 WW domain.Contains 1 ZZ-type zinc finger.