Differential Changes in Striatal Projection Neurons in R6/2 Transgenic Mice for Huntington's Disease

2002 ◽  
Vol 11 (3) ◽  
pp. 369-385 ◽  
Author(s):  
Z. Sun ◽  
N. Del Mar ◽  
C. Meade ◽  
D. Goldowitz ◽  
A. Reiner
Keyword(s):  
Transgenic Mice ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Projection Neurons ◽  
Striatal Projection Neurons

scholarly journals Striatal Potassium Channel Dysfunction in Huntington's Disease Transgenic Mice

2005 ◽  
Vol 93 (5) ◽  
pp. 2565-2574 ◽  
Author(s):  
Marjorie A. Ariano ◽  
Carlos Cepeda ◽  
Christopher R. Calvert ◽  
Jorge Flores-Hernández ◽  
Elizabeth Hernández-Echeagaray ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Mouse Models ◽  
Neurodegenerative Disorder ◽  
Projection Neurons ◽  
Electrophysiological Properties ◽  
Spiny Neurons ◽  
Subunit Expression ◽  
Dissociated Cells

Huntington's disease (HD) is a neurodegenerative disorder that mainly affects the projection neurons of the striatum and cerebral cortex. Genetic mouse models of HD have shown that neurons susceptible to the mutation exhibit morphological and electrophysiological dysfunctions before and during development of the behavioral phenotype. We used HD transgenic mouse models to examine inwardly and outwardly rectifying K+ conductances, as well as expression of some related K+ channel subunits. Experiments were conducted in slices and dissociated cells from two mouse models, the R6/2 and TgCAG100, at the beginning and after full development of overt behavioral phenotypes. Striatal medium-sized spiny neurons (MSNs) from symptomatic transgenic mice had increased input resistances, depolarized resting membrane potentials, and reductions in both inwardly and outwardly rectifying K+ currents. These changes were more dramatic in the R6/2 model than in the TgCAG100. Parallel immunofluorescence studies detected decreases in the expression of K+ channel subunit proteins, Kir2.1, Kir2.3, and Kv2.1 in MSNs, which contribute to the formation of the channel ionophores for these currents. Attenuation in K+ conductances and channel subunit expression contribute to altered electrophysiological properties of MSNs and may partially account for selective cellular vulnerability in the striatum.

Article Commentary: Development of the Human Striatum: Implications for Fetal Striatal Transplantation in the Treatment of Huntington's Disease

1995 ◽  
Vol 4 (6) ◽  
pp. 539-545
Author(s):  
Thomas B. Freeman ◽  
Paul R. Sanberg ◽  
Ole Isacson
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Fetal Tissue ◽  
The Body ◽  
Projection Neurons ◽  
Foramen Of Monro ◽  
Behavioral Deficits ◽  
Fetal Tissue Transplantation ◽  
Striatal Projection Neurons ◽  
Graft Success

Fetal neural transplantation has recently been demonstrated to ameliorate motor and other behavioral deficits in animal models of Huntington's disease, and reconstruct many of the damaged striatal circuits. However, there has been significant variability in the histological appearance of these grafts, most likely related to differences of the regions of dissection of the donor tissue. Selective dissection and transplantation of the lateral ventricular eminence in rodents has resulted in grafts consisting of primarily striatal-like tissue. This data, combined with data from our own and other laboratories has led to a description of the development of the human striatum, with a particular emphasis on the relevance of human striatal development to the field of fetal tissue transplantation for the treatment of Huntington's disease. If the goal of transplantation is to graft GABAergic striatal projection neurons, it is our impression that optimal grafting results will occur when transplants are derived from the lateral ventricular eminence and the lateral aspect of the body of the ventricular eminence anterior to the foramen of Monro. Optimal results are likely to occur when donor ages range from Stage 19 to 23, with possible graft success when donor age extends to as late as postovulatory week 22.

Abnormalities of Striatal Projection Neurons andN-Methyl-D-Aspartate Receptors in Presymptomatic Huntington's Disease

1990 ◽  
Vol 322 (18) ◽  
pp. 1293-1298 ◽  
Author(s):  
Roger L. Albin ◽  
Anne B. Young ◽  
John B. Penney ◽  
Barbara Handelin ◽  
Rosemary Balfour ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Projection Neurons ◽  
Striatal Projection Neurons

Neurturin protects striatal projection neurons but not interneurons in a rat model of Huntington’s disease

Neuroscience ◽  
2000 ◽  
Vol 98 (1) ◽  
pp. 89-96 ◽  
Author(s):  
E Pérez-Navarro ◽  
P Åkerud ◽  
S Marco ◽  
J.M Canals ◽  
E Tolosa ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rat Model ◽  
Projection Neurons ◽  
Striatal Projection Neurons
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