| The RabGEF ALS2 is a hypoxia inducible target associated with the acquisition of aggressive traits in tumor cells. | The RabGEF ALS2 is a hypoxia inducible target associated with the acquisition of aggressive traits in tumor cells.
Rivas S, Silva P, Reyes M, Sepúlveda H, Solano L, Acuña J, Guerrero M, Varas-Godoy M, Quest AFG, Montecino M, Torres VA., Free PMC Article | 05/8/2021 |
| two ALS-linked factors, SQSTM1 and ALS2, have distinct but additive protective roles against mutant SOD1-mediated toxicity by modulating neuronal proteostasis possibly through the autophagy-endolysosomal system. | Functional links between SQSTM1 and ALS2 in the pathogenesis of ALS: cumulative impact on the protection against mutant SOD1-mediated motor dysfunction in mice.
Hadano S, Mitsui S, Pan L, Otomo A, Kubo M, Sato K, Ono S, Onodera W, Abe K, Chen X, Koike M, Uchiyama Y, Aoki M, Warabi E, Yamamoto M, Ishii T, Yanagawa T, Shang HF, Yoshii F. | 06/24/2017 |
| By taking advantage of this CSMN reporter line of AlsinKO, authors reveal that apical dendrite degeneration is a common cellular pathology for CSMN that become diseased for different causes. | Absence of alsin function leads to corticospinal motor neuron vulnerability via novel disease mechanisms.
Gautam M, Jara JH, Sekerkova G, Yasvoina MV, Martina M, Özdinler PH., Free PMC Article | 12/17/2016 |
| alsin and spartin may interact each other physically. | Are alsin and spartin novel interaction partners?
Çobanoğlu G, Ozansoy M, Başak AN. | 03/2/2013 |
| Alsin and SOD1(G93A) proteins regulate endosomal reactive oxygen species production by glial cells and proinflammatory pathways responsible for neurotoxicity. | Alsin and SOD1(G93A) proteins regulate endosomal reactive oxygen species production by glial cells and proinflammatory pathways responsible for neurotoxicity.
Li Q, Spencer NY, Pantazis NJ, Engelhardt JF., Free PMC Article | 01/7/2012 |
| these results suggest that Als2 is a binding partner of Uxt and Als2/Uxt interaction could be important for the activation of Nf-kappaB pathway. | Alfa-class prefoldin protein UXT is a novel interacting partner of Amyotrophic Lateral Sclerosis 2 (Als2) protein.
Enunlu I, Ozansoy M, Basak AN. | 12/10/2011 |
| Rab5-mediated endocytosis was severely altered in ALS2(-/-) neurons. | Regulation of endosomal motility and degradation by amyotrophic lateral sclerosis 2/alsin.
Lai C, Xie C, Shim H, Chandran J, Howell BW, Cai H., Free PMC Article | 05/7/2011 |
| ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal trafficking | Loss of ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal trafficking.
Hadano S, Otomo A, Kunita R, Suzuki-Utsunomiya K, Akatsuka A, Koike M, Aoki M, Uchiyama Y, Itoyama Y, Ikeda JE., Free PMC Article | 01/15/2011 |
| Als2(-/-) mice showed a significantly lower spontaneous rearing activity than wild-type litters. These genetic background- and/or gender-specific findings suggest the presence of modifiers for life span and motor activities in Als2(-/-) mice. | Genetic background and gender effects on gross phenotypes in congenic lines of ALS2/alsin-deficient mice.
Hadano S, Yoshii Y, Otomo A, Kunita R, Suzuki-Utsunomiya K, Pan L, Kakuta S, Iwasaki Y, Iwakura Y, Ikeda JE. | 01/1/2011 |
| Als2-depleted spinal motor neurons can be rescued by co-cultured astrocytes. | Astrocytic protection of spinal motor neurons but not cortical neurons against loss of Als2/alsin function.
Jacquier A, Bellouze S, Blanchard S, Bohl D, Haase G. | 01/21/2010 |
| The Als2(-/-) mice lacking exon 2 and part of exon 3 developed mild signs of neurodegeneration compatible with axonal transport deficiency. | Als2 mRNA splicing variants detected in KO mice rescue severe motor dysfunction phenotype in Als2 knock-down zebrafish.
Gros-Louis F, Kriz J, Kabashi E, McDearmid J, Millecamps S, Urushitani M, Lin L, Dion P, Zhu Q, Drapeau P, Julien JP, Rouleau GA. | 01/21/2010 |
| ALS2 may act as a modulator in neuronal differentiation and/or development through regulation of membrane dynamics. | ALS2/alsin deficiency in neurons leads to mild defects in macropinocytosis and axonal growth.
Otomo A, Kunita R, Suzuki-Utsunomiya K, Mizumura H, Onoe K, Osuga H, Hadano S, Ikeda JE. | 01/21/2010 |
| alsin-deficient mice have motor impairment and degenerative pathology in the distal corticospinal tracts without apparent motor neuron pathology | Distal axonopathy in an alsin-deficient mouse model.
Deng HX, Zhai H, Fu R, Shi Y, Gorrie GH, Yang Y, Liu E, Dal Canto MC, Mugnaini E, Siddique T. | 01/21/2010 |
| Mouse models fail to recapitulate hallmarks of motor neuron disease, but the subtle deficits are observed in behavior and pathology. [RREVIEW] | Alsin and the molecular pathways of amyotrophic lateral sclerosis.
Chandran J, Ding J, Cai H., Free PMC Article | 01/21/2010 |
| Our data suggest that deficiency in the ALS2 gene does not affect the pathogenesis of SOD1(G93A) mice. | Deficiency in the ALS2 gene does not affect the motor neuron degeneration in SOD1(G93A) transgenic mice.
Lin X, Shim H, Cai H., Free PMC Article | 01/21/2010 |
| Novel function of ALS2 gene in lymphopoiesis and hematopoiesis, suggesting that immune system is involved in pathogenesis of one form of juvenile onset autosomal recessive amyotrophic lateral sclerosis(ALS2). | Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function.
Erie EA, Shim H, Smith AL, Lin X, Keyvanfar K, Xie C, Chen J, Cai H., Free PMC Article | 01/21/2010 |
| ALS2 is a novel Rac1 effector and is involved in Rac1-activated macropinocytosis. | The Rab5 activator ALS2/alsin acts as a novel Rac1 effector through Rac1-activated endocytosis.
Kunita R, Otomo A, Mizumura H, Suzuki-Utsunomiya K, Hadano S, Ikeda JE. | 01/21/2010 |
| alsin controls the growth and survival of motoneurons in a Rac1-dependant manner | Alsin/Rac1 signaling controls survival and growth of spinal motoneurons.
Jacquier A, Buhler E, Schäfer MK, Bohl D, Blanchard S, Beclin C, Haase G. | 01/21/2010 |
| deficiency in ALS2 causes an upper motor neuron disease that closely resembles a severe form of hereditary spastic paralysis, and that is quite distinct from amyotrophic lateral sclerosis. | Progressive spinal axonal degeneration and slowness in ALS2-deficient mice.
Yamanaka K, Miller TM, McAlonis-Downes M, Chun SJ, Cleveland DW. | 01/21/2010 |
| Rac1, PI3 kinase, and Akt3 have roles in an anti-apoptotic pathway triggered by ALS2 that antagonizes SOD1 mutant-induced motoneuronal cell death | A Rac1/phosphatidylinositol 3-kinase/Akt3 anti-apoptotic pathway, triggered by AlsinLF, the product of the ALS2 gene, antagonizes Cu/Zn-superoxide dismutase (SOD1) mutant-induced motoneuronal cell death.
Kanekura K, Hashimoto Y, Kita Y, Sasabe J, Aiso S, Nishimoto I, Matsuoka M. | 01/21/2010 |
| loss of ALS2 function is not sufficient to cause motor neuron disease in a mouse model. However, lack of ALS2 did predispose neurons to oxidative stress, implying that ALS2 might serve as a risk factor for motor neuron disease. | Loss of ALS2 function is insufficient to trigger motor neuron degeneration in knock-out mice but predisposes neurons to oxidative stress.
Cai H, Lin X, Xie C, Laird FM, Lai C, Wen H, Chiang HC, Shim H, Farah MH, Hoke A, Price DL, Wong PC., Free PMC Article | 01/21/2010 |
| The homozygous deletion in exon 4 of the ALS2 gene (553delA). | Novel mutation in the ALS2 gene in juvenile amyotrophic lateral sclerosis.
Kress JA, Kühnlein P, Winter P, Ludolph AC, Kassubek J, Müller U, Sperfeld AD. | 01/21/2010 |
| Alsin is a Rab5 and Rac1 guanine nucleotide exchange factor | Alsin is a Rab5 and Rac1 guanine nucleotide exchange factor.
Topp JD, Gray NW, Gerard RD, Horazdovsky BF. | 01/21/2010 |
| Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking. | Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking.
Hadano S, Benn SC, Kakuta S, Otomo A, Sudo K, Kunita R, Suzuki-Utsunomiya K, Mizumura H, Shefner JM, Cox GA, Iwakura Y, Brown RH Jr, Ikeda JE. | 01/21/2010 |
| Altered trophic receptor trafficking in neurons of Als2(-/-) mice may underlie the histopathological and behavioral changes observed and the pathogenesis of ALS2 | Als2-deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities.
Devon RS, Orban PC, Gerrow K, Barbieri MA, Schwab C, Cao LP, Helm JR, Bissada N, Cruz-Aguado R, Davidson TL, Witmer J, Metzler M, Lam CK, Tetzlaff W, Simpson EM, McCaffery JM, El-Husseini AE, Leavitt BR, Hayden MR., Free PMC Article | 01/21/2010 |