utrophin; dystrophin-related protein 1; DRP1; DRP (UTRN, DMDL)
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Function
- may play a role in anchoring the cytoskeleton to the plasma membrane
- interacts with the syntrophins SNTA1; SNTB1 & SNTB2
- interacts with SYNM
- may act as a functional endogenous replacement for dystrophin[3]
Structure
- contains 2 CH (calponin-homology) domains
- contains 20 spectrin repeats
- contains 1 WW domain
- contains 1 ZZ-type Zn+2 finger
Compartment
- cell junction, synapse, postsynaptic cell membrane
- peripheral membrane protein; cytoplasmic side
- cytoplasm, cytoskeleton
- neuromuscular junction
Expression
muscle
Pharmacology
- ezutromid, a utrophin modulator, is an investigative agent for treatment of children with Duchenne muscular dystrophy[3]
More general terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/P46939.html
- ↑ Wikipedia; Note: Utrophin entry http://en.wikipedia.org/wiki/Utrophin
- ↑ 3.0 3.1 3.2 George J, Ezutromid Shows Potential in DMD. Phase II study indicates modulating utrophin may reduce muscle inflammation. MedPage Today. April 29, 2018 https://www.medpagetoday.com/meetingcoverage/aan/72591
Muntoni F, et al Ezutromid significantly reduced muscle damage whilst maintaining utrophin in patients with Duchenne muscular dystrophy (DMD) after 24-weeks of treatment. Anerican Academy of Neurology (AAN) 2018.