Some of publications that our research group found interesting in the last month:

1: Cell Death Differ
Mutant SOD1 detoxification mechanisms in intact single cells.
http://www.als.net/articles/articleD...articleId=5310
PMID: 17992192 [PubMed - as supplied by publisher]

From John McCarty: Mutant SOD1 is known to cause ALS - but the actual mechanism this occurs is still a mystery. This article starts to answer the question of what is occurring in an individual cell with mutant SOD1. Ultimately, this question needs to be asked as well within the context of the intact spine, muscle and brain.


2: BMC Infect Dis
Expression of a Cu,Zn superoxide dismutase typical for familial amyotrophic lateral sclerosis increases the vulnerability of neuroblastoma cells to infectious injury.
http://www.als.net/articles/articleD...articleId=5309
PMID: 17997855 [PubMed - as supplied by publisher]

From John McCarty: Infection by virus or bacteria has been prominently considered as a possible environmental trigger or factor associated with ALS onset or progression. This paper suggests that mutant SOD1 may in fact increase the vulnerability of cells to infection and therefore would further emphasize early detection and treatment of respiratory infections in ALS.


3: Med Oral Patol Oral Cir Bucal
Salivary gland application of botulinum toxin for the treatment of sialorrhea.
http://www.als.net/articles/articleD...articleId=5308
PMID: 17978775 [PubMed - as supplied by publisher]

From John McCarty: Excessive saliva is a common quality of life associated problem in ALS. Botox toxin injections into the salivary glands is the subject of a trial in the US and has been anecdotally reported by a number of patients to help significantly with this issue.


4: Lancet Neurol
Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trial.
http://www.als.net/articles/articleD...articleId=5307
PMID: 17980667 [PubMed - as supplied by publisher]

From John McCarty: Minocycline, an antibiotic, has been proposed as neuroprotective for a variety of neuron-degenerative diseases and was the subject of this large Phase III trial in ALS - based in part on its reported efficacy in the mouse ALS model. ALS TDI has repeatedly independently tested minocycline in the mouse model with no observed efficacy - and, consistently, only a trend towards increasing progression was observed in this human trial report - rather than benefit.


5: J Neuropathol Exp Neurol
Intrathecal delivery of hepatocyte growth factor from amyotrophic lateral sclerosis onset suppresses disease progression in rat amyotrophic lateral sclerosis model.
http://www.als.net/articles/articleD...articleId=5306
PMID: 17984685 [PubMed - as supplied by publisher]

From John McCarty: Hepatocyte growth factor (HGF) is another potential neuroprotective protein that has growing evidence to support its possible benefit in the ALS animal model.


6: BMC Infect Dis
Amyotrophic lateral sclerosis and soccer: A different epidemiological approach strengthen the previous findings.
PMID: 17996254 [PubMed - as supplied by publisher]

From John McCarty: There continues to grow the conclusion that certain activities - such as Soccor in Europe - leads to increased risk of ALS. Uncertain would be whether this is due to specific exposures or would be inherent with the activity itself.


7: J Comp Neurol
Strong P2X(4) purinergic receptor-like immunoreactivity is selectively associated with degenerating neurons in transgenic rodent models of amyotrophic lateral sclerosis.
http://www.als.net/articles/articleD...articleId=5304
PMID: 17990272 [PubMed - as supplied by publisher]

From John McCarty: The brain is often not the focus of investigation in ALS - including in the animal models. This paper is one example where possible changes in non-motor neurons may be occurring as well.


8: J Neurosci
Exogenous delivery of heat shock protein 70 increases lifespan in a mouse model of amyotrophic lateral sclerosis.
http://www.als.net/articles/articleD...articleId=5299
PMID: 18045911 [PubMed - as supplied by publisher]

From John McCarty: This paper presents a new way to consider a potential role for chaperones - in this case HSP70 - in the pathology and potential treatment for ALS. While there has been significant emphasis on neuroprotective factors possibly playing a role in supporting the neuronal connection with muscles, there has not been much emphasis on the possible need for chaperones in the extra-cellular environment for their proper function.


9: PLoS ONE
Evidence of Compromised Blood-Spinal Cord Barrier in Early and Late Symptomatic SOD1 Mice Modeling ALS.
http://www.als.net/articles/articleD...articleId=5298
PMID: 18030339 [PubMed - as supplied by publisher]

From John McCarty: This open source article continues from an earlier paper from this same group describing defects observed in the animal in the protective sheath surrounding the central nervous system. As with any such observation associated with disease, the central question is whether the defect actually contributes to the process of disease development in the mouse model of if it is more a consequence of the active disease progression.


10: Physiol. Genomics
Gene profiling of skeletal muscle in an amyotrophic lateral sclerosis mouse model.
PMID: 18000159 [PubMed - as supplied by publisher]

From John McCarty: Gene profiling (transcriptome) studies will increasingly help to point out relevant pathways to target with drugs. ALS TDI has currently the world's largest transcriptome database and is being used to guide our discovery biology.


John McCarty, PhD
Treatment Investigator,
ALS Therapy Development Institute