Neurobiology of Aging
Volume 28, Issue 10 , Pages 1628-1630 , October 2007

Deficiency in the ALS2 gene does not affect the motor neuron degeneration in SOD1G93A transgenic mice

Received 15 May 2006 ,Revised 7 July 2006 ,Accepted 17 July 2006.

References 

  1. Cai H, Lin X, Xie C, Laird FM, Lai C, Wen H, et al. Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knockout mice but predisposes neurons to oxidative stress. J Neurosci. 2005;25:7567–7574
  2. Cleveland DW, Rothstein JD. From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat Rev Neurosci. 2001;2:806–819
  3. Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, Alexander DD, et al. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science. 1995;264:1772–1775
  4. Hadano S, Benn SC, Kakuta S, Otomo A, Sudo K, Kunita R, et al. Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking. Hum Mol Genet. 2006;15:233–250
  5. Hadano S, Hand CK, Osuga H, Yanagisawa Y, Otomo A, Devon RS, et al. A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat Genet. 2001;29:166–173
  6. Hadano S, Otomo A, Suzuki-Utsunomiya K, Kunita R, Yanagisawa Y, Showguchi-Miyata J, et al. ALS2CL, the novel protein highly homologous to the carboxy-terminal half of ALS2, binds to Rab5 and modulates endosome dynamics. FEBS Lett. 2004;575:64–70
  7. Kanekura K, Hashimoto Y, Kita Y, Sasabe J, Aiso S, Nishimoto I, et al. A Rac1/phosphatidylinositol 3-kinase/Akt3 anti-apoptotic pathway, triggered by AlsinLF, the product of the ALS2 gene, antagonizes Cu/Zn-superoxide dismutase (SOD1) mutantinduced motoneuronal cell death. J Biol Chem. 2005;280:4532–4543
  8. Kieran D, Hafezparast M, Bohnert S, Dick JR, Martin J, Schiavo G, et al. A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice. J Cell Biol. 2005;169:561–567
  9. Yang Y, Hentati A, Deng HX, Dabbagh O, Sasaki T, Hirano M, et al. The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis. Nat Genet. 2001;29:160–165

PII: S0197-4580(06)00267-3

doi: 10.1016/j.neurobiolaging.2006.07.014

Neurobiology of Aging
Volume 28, Issue 10 , Pages 1628-1630 , October 2007

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