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PMID:16107644

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Citation

Cai, H, Lin, X, Xie, C, Laird, FM, Lai, C, Wen, H, Chiang, HC, Shim, H, Farah, MH, Hoke, A, Price, DL and Wong, PC (2005) Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knock-out mice but predisposes neurons to oxidative stress. J. Neurosci. 25:7567-74

Abstract

Amyotrophic lateral sclerosis (ALS), the most common motor neuron disease, is caused by a selective loss of motor neurons in the CNS. Mutations in the ALS2 gene have been linked to one form of autosomal recessive juvenile onset ALS (ALS2). To investigate the pathogenic mechanisms of ALS2, we generated ALS2 knock-out (ALS2(-/-)) mice. Although ALS2(-/-) mice lacked obvious developmental abnormalities, they exhibited age-dependent deficits in motor coordination and motor learning. Moreover, ALS2(-/-) mice showed a higher anxiety response in the open-field and elevated plus-maze tasks. Although they failed to recapitulate clinical or neuropathological phenotypes consistent with motor neuron disease by 20 months of age, ALS2(-/-) mice or primary cultured neurons derived from these mice were more susceptible to oxidative stress compared with wild-type controls. These observations suggest that loss of ALS2 function is insufficient to cause major motor deficits or motor neuron degeneration in a mouse model but predisposes neurons to oxidative stress.

Links

PubMed PMC2364727 Online version:10.1523/JNEUROSCI.1645-05.2005

Keywords

Amyotrophic Lateral Sclerosis/genetics; Amyotrophic Lateral Sclerosis/metabolism; Animals; Anxiety/genetics; Anxiety/metabolism; Anxiety/psychology; Cells, Cultured; Female; Guanine Nucleotide Exchange Factors/deficiency; Guanine Nucleotide Exchange Factors/genetics; Guanine Nucleotide Exchange Factors/physiology; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Motor Neurons/metabolism; Motor Skills/physiology; Nerve Degeneration/genetics; Nerve Degeneration/metabolism; Nerve Degeneration/psychology; Neurons/metabolism; Oxidative Stress/physiology

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

MOUSE:ALS2

acts_upstream_of_or_within

GO:0001662: behavioral fear response

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:3522476

P

Seeded From UniProt

complete

MOUSE:ALS2

acts_upstream_of_or_within

GO:0007626: locomotory behavior

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:3522476

P

Seeded From UniProt

complete

MOUSE:ALS2

acts_upstream_of_or_within

GO:0006979: response to oxidative stress

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:3522476

P

Seeded From UniProt

complete

See also

References

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