PMID- 29193204
OWN - NLM
STAT- MEDLINE
DCOM- 20190327
LR  - 20190501
IS  - 1097-4598 (Electronic)
IS  - 0148-639X (Print)
IS  - 0148-639X (Linking)
VI  - 57
IP  - 5
DP  - 2018 May
TI  - Engineered agrin attenuates the severity of experimental autoimmune myasthenia 
      gravis.
PG  - 814-820
LID - 10.1002/mus.26025 [doi]
AB  - INTRODUCTION: Agrin is essential for the formation and maintenance of 
      neuromuscular junctions (NMJs). NT-1654 is a C-terminal fragment of mouse neural 
      agrin. In this study, we determined the effects of NT-1654 on the severity of 
      experimental autoimmune myasthenia gravis (EAMG). METHODS: EAMG was induced in 
      female Lewis rats by immunization with the Torpedo acetylcholine receptor (tAChR) 
      and complete Freund's adjuvant (CFA). NT-1654 was dissolved in phosphate-buffered 
      saline (PBS) and injected daily subcutaneously into tAChR immunized rats during 
      the first 10 days after immunization, and then every other day for the following 
      20 days. RESULTS: We showed that NT-1654 attenuated clinical severity, 
      effectively promoted the clustering of AChRs at NMJs, and alleviated the 
      impairment of NMJ transmission and the reduction of muscle-specific kinase (MuSK) 
      in EAMG rats. DISCUSSION: We demonstrated that NT-1654 attenuated clinical 
      severity, effectively promoted the clustering of AChRs at NMJs, and alleviated 
      the impairment of NMJ transmission and the reduction of muscle-specific kinase 
      (MuSK) in EAMG rats. Muscle Nerve 57: 814-820, 2018.
CI  - (c) 2017 Wiley Periodicals, Inc.
FAU - Li, Zhiguo
AU  - Li Z
AD  - Department of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and 
      Medical Center, Phoenix, Arizona, 85013, USA.
AD  - Department of Neurology, Tianjin Neurological Institute, Tianjin Medical 
      University General Hospital, Tianjin, China.
FAU - Li, Minshu
AU  - Li M
AD  - Department of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and 
      Medical Center, Phoenix, Arizona, 85013, USA.
AD  - Department of Neurology, Tianjin Neurological Institute, Tianjin Medical 
      University General Hospital, Tianjin, China.
FAU - Wood, Kristofer
AU  - Wood K
AD  - Department of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and 
      Medical Center, Phoenix, Arizona, 85013, USA.
FAU - Hettwer, Steffan
AU  - Hettwer S
AD  - Neurotune AG, Schlieren-Zurich, Switzerland.
FAU - Muley, Suraj A
AU  - Muley SA
AD  - Department of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and 
      Medical Center, Phoenix, Arizona, 85013, USA.
FAU - Shi, Fu-Dong
AU  - Shi FD
AD  - Department of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and 
      Medical Center, Phoenix, Arizona, 85013, USA.
AD  - Department of Neurology, Tianjin Neurological Institute, Tianjin Medical 
      University General Hospital, Tianjin, China.
FAU - Liu, Qiang
AU  - Liu Q
AD  - Department of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and 
      Medical Center, Phoenix, Arizona, 85013, USA.
AD  - Department of Neurology, Tianjin Neurological Institute, Tianjin Medical 
      University General Hospital, Tianjin, China.
FAU - Ladha, Shafeeq S
AU  - Ladha SS
AD  - Department of Neurology, Barrow Neurological Institute, St. Joseph's Hospital and 
      Medical Center, Phoenix, Arizona, 85013, USA.
LA  - eng
GR  - R01 NS092713/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20180108
PL  - United States
TA  - Muscle Nerve
JT  - Muscle & nerve
JID - 7803146
RN  - 0 (Agrin)
RN  - 0 (Autoantibodies)
RN  - 0 (C-terminal agrin fragment)
RN  - 0 (Membrane Glycoproteins)
RN  - 0 (Nerve Tissue Proteins)
RN  - 0 (Neurofibromin 1)
RN  - 0 (Peptide Fragments)
RN  - 0 (Receptors, Cholinergic)
RN  - 0 (Sv2a protein, rat)
RN  - 9007-81-2 (Freund's Adjuvant)
SB  - IM
MH  - Action Potentials/physiology
MH  - Agrin/biosynthesis/chemistry/*therapeutic use
MH  - Animals
MH  - Autoantibodies/metabolism
MH  - Disease Models, Animal
MH  - Dose-Response Relationship, Drug
MH  - Electromyography
MH  - Female
MH  - Freund's Adjuvant/toxicity
MH  - Gene Expression Regulation/drug effects
MH  - Immunization/*adverse effects
MH  - Membrane Glycoproteins/metabolism
MH  - Muscle, Skeletal/pathology
MH  - Muscular Atrophy/etiology/therapy
MH  - Myasthenia Gravis, Autoimmune, Experimental/*drug therapy/*pathology
MH  - Nerve Tissue Proteins/metabolism
MH  - Neurofibromin 1/metabolism
MH  - Neuromuscular Junction/pathology
MH  - Peptide Fragments/biosynthesis/chemistry/*therapeutic use
MH  - Rats
MH  - Rats, Inbred Lew
MH  - Receptors, Cholinergic/immunology/metabolism
PMC - PMC5910282
MID - NIHMS923726
OTO - NOTNLM
OT  - acetylcholine receptor
OT  - agrin
OT  - myasthenia gravis
OT  - neuromuscular junctions
COIS- Disclosure of Conflicts of Interest: None of the authors has any conflict of 
      interest to disclose.
EDAT- 2017/12/02 06:00
MHDA- 2019/03/28 06:00
PMCR- 2019/05/01
CRDT- 2017/12/02 06:00
PHST- 2017/03/11 00:00 [received]
PHST- 2017/11/22 00:00 [revised]
PHST- 2017/11/26 00:00 [accepted]
PHST- 2017/12/02 06:00 [pubmed]
PHST- 2019/03/28 06:00 [medline]
PHST- 2017/12/02 06:00 [entrez]
PHST- 2019/05/01 00:00 [pmc-release]
AID - 10.1002/mus.26025 [doi]
PST - ppublish
SO  - Muscle Nerve. 2018 May;57(5):814-820. doi: 10.1002/mus.26025. Epub 2018 Jan 8.