PMID- 23508358
OWN - NLM
STAT- MEDLINE
DCOM- 20140212
LR  - 20211021
IS  - 1559-1182 (Electronic)
IS  - 0893-7648 (Linking)
VI  - 48
IP  - 1
DP  - 2013 Aug
TI  - Central nervous system involvement in the animal model of myodystrophy.
PG  - 71-7
LID - 10.1007/s12035-013-8415-9 [doi]
AB  - Congenital muscular dystrophies present mutated gene in the LARGE mice model and 
      it is characterized by an abnormal glycosylation of alpha-dystroglycan (alpha-DG), 
      strongly implicated as having a causative role in the development of central 
      nervous system abnormalities such as cognitive impairment seen in patients. 
      However, the pathophysiology of the brain involvement remains unclear. Therefore, 
      the objective of this study is to evaluate the oxidative damage and energetic 
      metabolism in the brain tissue as well as cognitive involvement in the 
      LARGE((myd)) mice model of muscular dystrophy. With this aim, we used adult 
      homozygous, heterozygous, and wild-type mice that were divided into two groups: 
      behavior and biochemical analyses. In summary, it was observed that homozygous 
      mice presented impairment to the habituation and avoidance memory tasks; low 
      levels of brain-derived neurotrophic factor (BDNF) in the prefrontal cortex, 
      hippocampus, cortex and cerebellum; increased lipid peroxidation in the 
      prefrontal cortex, hippocampus, striatum, and cerebellum; an increase of protein 
      peroxidation in the prefrontal cortex, hippocampus, striatum, cerebellum, and 
      cortex; a decrease of complex I activity in the prefrontal cortex and cerebellum; 
      a decrease of complex II activity in the prefrontal cortex and cerebellum; a 
      decrease of complex IV activity in the prefrontal cortex and cerebellum; an 
      increase in the cortex; and an increase of creatine kinase activity in the 
      striatum and cerebellum. This study shows the first evidence that abnormal 
      glycosylation of alpha-DG may be affecting BDNF levels, oxidative particles, and 
      energetic metabolism thus contributing to the memory storage and restoring 
      process.
FAU - Comim, Clarissa M
AU  - Comim CM
AD  - Laboratory of Experimental Neurosciences, University of Southern Santa Catarina, 
      88137-270, Palhoca, Santa Catarina, Brazil. clarissamc@terra.com.br
FAU - Mendonca, Bruna P
AU  - Mendonca BP
FAU - Dominguini, Diogo
AU  - Dominguini D
FAU - Cipriano, Andreza L
AU  - Cipriano AL
FAU - Steckert, Amanda V
AU  - Steckert AV
FAU - Scaini, Giselli
AU  - Scaini G
FAU - Vainzof, Mariz
AU  - Vainzof M
FAU - Streck, Emilio L
AU  - Streck EL
FAU - Dal-Pizzol, Felipe
AU  - Dal-Pizzol F
FAU - Quevedo, Joao
AU  - Quevedo J
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20130319
PL  - United States
TA  - Mol Neurobiol
JT  - Molecular neurobiology
JID - 8900963
RN  - 0 (Brain-Derived Neurotrophic Factor)
SB  - IM
MH  - Animals
MH  - Avoidance Learning
MH  - Behavior, Animal
MH  - Brain-Derived Neurotrophic Factor/metabolism
MH  - Central Nervous System/metabolism/*pathology
MH  - Disease Models, Animal
MH  - Electron Transport
MH  - Energy Metabolism
MH  - Mice
MH  - Muscular Dystrophies/metabolism/*pathology
MH  - Oxidation-Reduction
MH  - Oxidative Stress
EDAT- 2013/03/20 06:00
MHDA- 2014/02/13 06:00
CRDT- 2013/03/20 06:00
PHST- 2012/11/02 00:00 [received]
PHST- 2013/01/29 00:00 [accepted]
PHST- 2013/03/20 06:00 [entrez]
PHST- 2013/03/20 06:00 [pubmed]
PHST- 2014/02/13 06:00 [medline]
AID - 10.1007/s12035-013-8415-9 [doi]
PST - ppublish
SO  - Mol Neurobiol. 2013 Aug;48(1):71-7. doi: 10.1007/s12035-013-8415-9. Epub 2013 Mar 
      19.