PMID- 29023846
OWN - NLM
STAT- MEDLINE
DCOM- 20190305
LR  - 20190305
IS  - 1097-4598 (Electronic)
IS  - 0148-639X (Linking)
VI  - 57
IP  - 4
DP  - 2018 Apr
TI  - Locomotor and skeletal muscle abnormalities in trembler J neuropathic mice.
PG  - 664-671
LID - 10.1002/mus.25987 [doi]
AB  - INTRODUCTION: Patients with hereditary peripheral neuropathies exhibit 
      characteristic deformities of the hands and feet and have difficulty ambulating. 
      To examine to what extent neuropathic animals recapitulate these deficits, we 
      studied trembler J (TrJ) mice, which model early-onset demyelinating neuropathy. 
      METHODS: A cohort of 4-month-old female wild type and neuropathic mice were 
      evaluated for locomotor measurements, neuromuscular function, and skeletal muscle 
      proteolysis and morphometry. RESULTS: Utilizing the DigiGait imaging system, we 
      identified pronounced alterations in forepaw and hindpaw angles and a decrease in 
      hindpaw area on the treadmill in neuropathic rodents. Torque production by the 
      tibialis anterior (TA) muscle was significantly weakened and was paralleled by a 
      decrease in myofiber cross-sectional area and an increase in muscle tissue 
      proteolysis. DISCUSSION: Our findings in TrJ mice reflect the phenotypic 
      presentation of the human neuropathy in which patients exhibit weakness of the TA 
      muscle resulting in foot drop and locomotor abnormalities. Muscle Nerve 57: 
      664-671, 2018.
CI  - (c) 2017 Wiley Periodicals, Inc.
FAU - Falk, Darin J
AU  - Falk DJ
AD  - Department of Neuroscience, College of Medicine, McKnight Brain Institute, 
      University of Florida, 1149 Newell Drive, Box 100244 Gainesville, Florida, 
      32610-0244, USA.
AD  - Department of Pediatrics, College of Medicine, University of Florida, 
      Gainesville, Florida, 32610-0244, USA.
FAU - Galatas, Tori
AU  - Galatas T
AD  - Department of Neuroscience, College of Medicine, McKnight Brain Institute, 
      University of Florida, 1149 Newell Drive, Box 100244 Gainesville, Florida, 
      32610-0244, USA.
FAU - Todd, Adrian G
AU  - Todd AG
AD  - Department of Pediatrics, College of Medicine, University of Florida, 
      Gainesville, Florida, 32610-0244, USA.
FAU - Soto, Elliott P
AU  - Soto EP
AD  - Department of Neuroscience, College of Medicine, McKnight Brain Institute, 
      University of Florida, 1149 Newell Drive, Box 100244 Gainesville, Florida, 
      32610-0244, USA.
FAU - Harris, Andrew B
AU  - Harris AB
AD  - Department of Neuroscience, College of Medicine, McKnight Brain Institute, 
      University of Florida, 1149 Newell Drive, Box 100244 Gainesville, Florida, 
      32610-0244, USA.
FAU - Notterpek, Lucia
AU  - Notterpek L
AD  - Department of Neuroscience, College of Medicine, McKnight Brain Institute, 
      University of Florida, 1149 Newell Drive, Box 100244 Gainesville, Florida, 
      32610-0244, USA.
AD  - Department of Neurology, College of Medicine, McKnight Brain Institute, 
      University of Florida, Gainesville, Florida, USA.
LA  - eng
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
DEP - 20171024
PL  - United States
TA  - Muscle Nerve
JT  - Muscle & nerve
JID - 7803146
RN  - 0 (Myelin Proteins)
RN  - 0 (Pmp22 protein, mouse)
SB  - IM
MH  - Animals
MH  - Charcot-Marie-Tooth Disease/genetics/*physiopathology
MH  - Disease Models, Animal
MH  - Female
MH  - Gait Analysis
MH  - Hereditary Sensory and Motor Neuropathy/genetics/physiopathology
MH  - Locomotion/*physiology
MH  - Mice
MH  - Muscle, Skeletal/*physiopathology
MH  - Myelin Proteins/genetics
MH  - Peripheral Nervous System Diseases/genetics/physiopathology
MH  - Phenotype
MH  - Torque
OTO - NOTNLM
OT  - Charcot-Marie-Tooth disease
OT  - demyelination
OT  - muscle atrophy
OT  - neuromuscular function
OT  - neuropathy
EDAT- 2017/10/13 06:00
MHDA- 2019/03/06 06:00
CRDT- 2017/10/13 06:00
PHST- 2017/10/07 00:00 [accepted]
PHST- 2017/10/13 06:00 [pubmed]
PHST- 2019/03/06 06:00 [medline]
PHST- 2017/10/13 06:00 [entrez]
AID - 10.1002/mus.25987 [doi]
PST - ppublish
SO  - Muscle Nerve. 2018 Apr;57(4):664-671. doi: 10.1002/mus.25987. Epub 2017 Oct 24.