RESEARCH: Drug delays progression in ALS mice

[Guest guest]

Treatment with arimoclomol, a coinducer of heat shock

proteins, delays disease progression in ALS mice.

Kieran D, Kalmar B, Dick JR, Riddoch-Contreras J,

Burnstock G, Greensmith L.

The Graham Watts Laboratory, Sobell Department of

Motor Neuroscience and Movement Disorders, Institute

of Neurology, The National Hospital for Neurology and

Neurosurgery, University College London, Queen Square,

London WC1N 3BG, UK.

Amyotrophic lateral sclerosis (ALS) is a fatal

neurodegenerative condition in which motoneurons of

the spinal cord and motor cortex die, resulting in

progressive paralysis. This condition has no cure and

results in eventual death, usually within 1-5 years of

diagnosis. Although the specific etiology of ALS is

unknown, 20% of familial cases of the disease carry

mutations in the gene encoding Cu/Zn superoxide

dismutase-1 (SOD1). Transgenic mice overexpressing

human mutant SOD1 have a phenotype and pathology that

are very similar to that seen in human ALS patients.

Here we show that treatment with arimoclomol, a

coinducer of heat shock proteins (HSPs), significantly

delays disease progression in mice expressing a SOD1

mutant in which glycine is substituted with alanine at

position 93 (SOD1(G93A)). Arimoclomol-treated

SOD1(G93A) mice show marked improvement in hind limb

muscle function and motoneuron survival in the later

stages of the disease, resulting in a 22% increase in

lifespan. Pharmacological activation of the heat shock

response may therefore be a successful therapeutic

approach to treating ALS, and possibly other

neurodegenerative diseases.

SOURCE: Nat Med. 2004 Apr;10(4):402-5. Epub 2004 Mar

21.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve & db=PubMed & list_uids=1\

5034571 & dopt=Abstract

[Guest guest]

Yep, closer and closer!! Someday soon guys, someday soon we will have the

victory over all neurological diseases. Thanks for the update Mark.

God Bless

Yolanda

RESEARCH: Drug delays progression in ALS mice

Treatment with arimoclomol, a coinducer of heat shock

proteins, delays disease progression in ALS mice.

Kieran D, Kalmar B, Dick JR, Riddoch-Contreras J,

Burnstock G, Greensmith L.

The Graham Watts Laboratory, Sobell Department of

Motor Neuroscience and Movement Disorders, Institute

of Neurology, The National Hospital for Neurology and

Neurosurgery, University College London, Queen Square,

London WC1N 3BG, UK.

Amyotrophic lateral sclerosis (ALS) is a fatal

neurodegenerative condition in which motoneurons of

the spinal cord and motor cortex die, resulting in

progressive paralysis. This condition has no cure and

results in eventual death, usually within 1-5 years of

diagnosis. Although the specific etiology of ALS is

unknown, 20% of familial cases of the disease carry

mutations in the gene encoding Cu/Zn superoxide

dismutase-1 (SOD1). Transgenic mice overexpressing

human mutant SOD1 have a phenotype and pathology that

are very similar to that seen in human ALS patients.

Here we show that treatment with arimoclomol, a

coinducer of heat shock proteins (HSPs), significantly

delays disease progression in mice expressing a SOD1

mutant in which glycine is substituted with alanine at

position 93 (SOD1(G93A)). Arimoclomol-treated

SOD1(G93A) mice show marked improvement in hind limb

muscle function and motoneuron survival in the later

stages of the disease, resulting in a 22% increase in

lifespan. Pharmacological activation of the heat shock

response may therefore be a successful therapeutic

approach to treating ALS, and possibly other

neurodegenerative diseases.

SOURCE: Nat Med. 2004 Apr;10(4):402-5. Epub 2004 Mar

21.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve & db=PubMed & list_uids=1\

5034571 & dopt=Abstract