The use of transgenic mouse models of amyotrophic lateral sclerosis in preclinical drug studies (Q41674461)

6 October 2017

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title

The use of transgenic mouse models of amyotrophic lateral sclerosis in preclinical drug studies (English)

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amyotrophic lateral sclerosis

6 October 2017

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author name string

Gurney ME

series ordinal

1

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6 October 2017

1 October 1997

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Journal of the Neurological Sciences

6 October 2017

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6 October 2017

152 Suppl 1

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6 October 2017

S67-73

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Amyotrophic lateral sclerosis associated with homozygosity for an Asp90Ala mutation in CuZn-superoxide dismutase

6 October 2017

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Progressive motor neuron impairment in an animal model of familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

ALS, SOD and peroxynitrite

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Superoxide dismutase 1 with mutations linked to familial amyotrophic lateral sclerosis possesses significant activity

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Defective axonal transport in a transgenic mouse model of amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Neuropathological changes in two lines of mice carrying a transgene for mutant human Cu,Zn SOD, and in mice overexpressing wild type human SOD: a model of familial amyotrophic lateral sclerosis (FALS).

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Amyotrophic lateral sclerosis and structural defects in Cu,Zn superoxide dismutase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

An hereditary motor neurone disease with progressive denervation of muscle in the mouse: the mutant 'wobbler'.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Faster superoxide dismutase mutants designed by enhancing electrostatic guidance

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Familial amyotrophic lateral sclerosis. A subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Interaction of bovine erythrocyte superoxide dismutase with hydrogen peroxide. Inactivation of the enzyme

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Midbrain dopaminergic neuronal degeneration in a transgenic mouse model of familial amyotrophic lateral sclerosis.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Mutations in copper-zinc superoxide dismutase that cause amyotrophic lateral sclerosis alter the zinc binding site and the redox behavior of the protein

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Arrest of motor neuron disease in wobbler mice cotreated with CNTF and BDNF

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

The Golgi apparatus of spinal cord motor neurons in transgenic mice expressing mutant Cu,Zn superoxide dismutase becomes fragmented in early, preclinical stages of the disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Excitatory amino acid release and free radical formation may cooperate in the genesis of ischemia-induced neuronal damage.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Transgenic mice expressing an altered murine superoxide dismutase gene provide an animal model of amyotrophic lateral sclerosis

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

GDNF slows loss of motoneurons but not axonal degeneration or premature death of pmn/pmn mice.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

A new mouse mutant with progressive motor neuronopathy.

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Ciliary neurotrophic factor prevents degeneration of motor neurons in mouse mutant progressive motor neuronopathy

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Genetics of amyotrophic lateral sclerosis

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Linkage of a gene causing familial amyotrophic lateral sclerosis to chromosome 21 and evidence of genetic-locus heterogeneity

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Altered reactivity of superoxide dismutase in familial amyotrophic lateral sclerosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Copper, zinc superoxide dismutase catalyzes hydroxyl radical production from hydrogen peroxide

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

A gain-of-function of an amyotrophic lateral sclerosis-associated Cu,Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0022-510X%2897%2900247-5

10.1016/S0022-510X(97)00247-5

7 January 2021

Identifiers

DOI

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6 October 2017

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PubMed ID

9419057