+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ PDF Full Text
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn

+ Translate
+ Recently Requested

Trials of stem cell transplantation in the transgenic SOD1 ALS mice

Trials of stem cell transplantation in the transgenic SOD1 ALS mice

Society for Neuroscience Abstract Viewer & Itinerary Planner : Abstract No 495 15

Death of motor neurons causes weakness, respiratory paralysis and death in amyotrophic lateral sclerosis (ALS). Since it can potentially replace lost motor neurons or enhance motor neuron viability, cell-based therapy is being explored in ALS. This approach is strengthened by the recent observation that a relatively small sub-population (20%) of wild-type cells can rescue motor neuron cell death in chimeric SOD1WT-SOD1G93A mice. In a recent study, transplantation of neuronal progenitor hNT cells (human teratocarcinonma cell-line Ntera 2/D1) into the spinal cord of SOD1G93A transgenic ALS mice increased survival and improved motor function. In several studies, we have investigated the effect of human stem cells on the disease onset and survival of SOD1G93A transgenic ALS mice, following either systemic intravenous or intraspinal transplantation. Using human fetal neural stem cells, we observe a delay in disease onset (13 +/-5.7 days, p=0.0003) and increase in survival (16 days +/-6.0 days, p<0.0001) compared to control cell treated mice following intravenous administration of cells, and a survival benefit of 9 days +/-7.0 days after multi-site intraparenchymal delivery. Immunohistochemical analyses of tissue from treated ALS mice identify transplanted cells in the spinal cord at the terminal stage of disease.

(PDF emailed within 1 workday: $29.90)

Accession: 035985927

Download citation: RISBibTeXText

Related references

Functional neural stem cell isolation from brains of adult mutant SOD1 (SOD1(G93A)) transgenic amyotrophic lateral sclerosis (ALS) mice. Neurological Research 33(1): 33-37, 2011

Advanced glycation endproduct-modified superoxide dismutase-1 (SOD1)-positive inclusions are common to familial amyotrophic lateral sclerosis patients with SOD1 gene mutations and transgenic mice expressing human SOD1 with a G85R mutation. Acta Neuropathologica 100(5): 490-505, 2000

Transplanted neural stem cells are capable of engraftment and differentiation in transgenic mutant SOD1 mice. Society for Neuroscience Abstracts 26(1-2): Abstract No -668 3, 2000

SOD1 immunoreactive precipitates in mice transgenic for human SOD1 with and without mutations. Society for Neuroscience Abstracts 25(1-2): 1835, 1999

Exploratory activity and motor coordination in wild-type SOD1/SOD1 transgenic mice. Brain Research Bulletin 66(2): 155-162, 2005

Swelling and vacuolisation of mitochondria in transgenic SOD1-ALS mice: a consequence of supranormal SOD1 expression?. Mitochondrion 6(1): 48-9; Author Reply 50-1, 2006

Rescue of anaemia and autoimmune responses in SOD1-deficient mice by transgenic expression of human SOD1 in erythrocytes. Biochemical Journal 422(2): 313-320, 2009

Wild type SOD1 promotes non-disease transgenic mice expressing A4V-SOD1 develop ALS phenotype and pathology. Society for Neuroscience Abstracts 27(2): 2343, 2001

Motor neuron disease is not ameliorated in mutant SOD1 transgenic mice deficient in copper chaperone for SOD1. Society for Neuroscience Abstracts 26(1-2): Abstract No -182 14, 2000

Copper-binding-site-null SOD1 causes ALS in transgenic mice: aggregates of non-native SOD1 delineate a common feature. Human Molecular Genetics 12(21): 2753-2764, 2003

Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice. Human Molecular Genetics 24(4): 1019-1035, 2015

Reporting of adverse event data in hematopoietic stem cell transplantation clinical trials involving investigational new drugs or devices: a report from the William Guy Forbeck Foundation 2001 focus meeting on clinical trials in hematopoietic stem cell transplantation. Biology of Blood and Marrow Transplantation 8(6): 295-302, 2002

Decreased GLT-1 and increased SOD1 and HO-1 expression in astrocytes contribute to lumbar spinal cord vulnerability of SOD1-G93A transgenic mice. Febs Letters 584(8): 1615-1622, 2010

Dual transplantation of human neural stem cells into cervical and lumbar cord ameliorates motor neuron disease in SOD1 transgenic rats. Neuroscience Letters 494(3): 222-226, 2011