Administration of Recombinant Heat Shock Protein 70 Delays Peripheral Muscle Denervation in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis
Joint Authors
Rouhani, Hesam
Milligan, Carol
Gifondorwa, David J.
Caress, James
Jimenz-Moreno, Ramon
Robinson, Mac B.
Strupe, Jane L.
Hayes, Crystal D.
Source
Neurology Research International
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-14, 14 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-08-01
Country of Publication
Egypt
No. of Pages
14
Main Subjects
Abstract EN
A prominent clinical feature of ALS is muscle weakness due to dysfunction, denervation and degeneration of motoneurons (MNs).
While MN degeneration is a late stage event in the ALS mouse model, muscle denervation occurs significantly earlier in the disease.
Strategies to prevent this early denervation may improve quality of life by maintaining muscle control and slowing disease progression.
The precise cause of MN dysfunction and denervation is not known, but several mechanisms have been proposed that involve potentially toxic intra- and extracellular changes.
Many cells confront these changes by mounting a stress response that includes increased expression of heat shock protein 70 (Hsp70).
MNs do not upregulate Hsp70, and this may result in a potentially increased vulnerability.
We previously reported that recombinant human hsp70 (rhHsp70) injections delayed symptom onset and increased lifespan in SOD1G93A mice.
The exogenous rhHsp70 was localized to the muscle and not to spinal cord or brain suggesting it modulates peripheral pathophysiology.
In the current study, we focused on earlier administration of Hsp70 and its effect on initial muscle denervation.
Injections of the protein appeared to arrest denervation with preserved large myelinated peripheral axons, and reduced glial activation.
American Psychological Association (APA)
Gifondorwa, David J.& Jimenz-Moreno, Ramon& Hayes, Crystal D.& Rouhani, Hesam& Robinson, Mac B.& Strupe, Jane L.…[et al.]. 2012. Administration of Recombinant Heat Shock Protein 70 Delays Peripheral Muscle Denervation in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis. Neurology Research International،Vol. 2012, no. 2012, pp.1-14.
https://search.emarefa.net/detail/BIM-451486
Modern Language Association (MLA)
Gifondorwa, David J.…[et al.]. Administration of Recombinant Heat Shock Protein 70 Delays Peripheral Muscle Denervation in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis. Neurology Research International No. 2012 (2012), pp.1-14.
https://search.emarefa.net/detail/BIM-451486
American Medical Association (AMA)
Gifondorwa, David J.& Jimenz-Moreno, Ramon& Hayes, Crystal D.& Rouhani, Hesam& Robinson, Mac B.& Strupe, Jane L.…[et al.]. Administration of Recombinant Heat Shock Protein 70 Delays Peripheral Muscle Denervation in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis. Neurology Research International. 2012. Vol. 2012, no. 2012, pp.1-14.
https://search.emarefa.net/detail/BIM-451486
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-451486