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View article: Central nervous system specific high molecular weight ALS2/alsin homophilic complex is enriched in mouse brain synaptosomes
Central nervous system specific high molecular weight ALS2/alsin homophilic complex is enriched in mouse brain synaptosomes Open
ALS2/alsin, the causative gene product for a number of juvenile recessive motor neuron diseases, acts as a guanine nucleotide exchange factor (GEF) for Rab5, regulating early endosome trafficking and maturation. It has been demonstrated th…
View article: Personalized Treatment for Infantile Ascending Hereditary Spastic Paralysis Based on In Silico Strategies
Personalized Treatment for Infantile Ascending Hereditary Spastic Paralysis Based on In Silico Strategies Open
Infantile onset hereditary spastic paralysis (IAHSP) is a rare neurological disease diagnosed in less than 50 children worldwide. It is transmitted with a recessive pattern and originates from mutations of the ALS2 gene, encoding for the p…
View article: SQSTM1, a protective factor of SOD1-linked motor neuron disease, regulates the accumulation and distribution of ubiquitinated protein aggregates in neuron
SQSTM1, a protective factor of SOD1-linked motor neuron disease, regulates the accumulation and distribution of ubiquitinated protein aggregates in neuron Open
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective loss of motor neurons in the brain and spinal cord. Recent studies have shown that mutations in SQSTM1 are linked to ALS. It has also…
View article: High-throughput quantitative analysis of axonal transport in cultured neurons from SOD1H46R ALS mice by using a microfluidic device
High-throughput quantitative analysis of axonal transport in cultured neurons from SOD1H46R ALS mice by using a microfluidic device Open
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective loss of motor neurons. We have previously shown that autophagosome-like vesicular structures are progressively accumulated in the spinal axons of…
View article: Development of microsatellite markers for the geographically parthenogenetic stick insect <i>Phraortes elongatus</i> (Insecta: Phasmatodea)
Development of microsatellite markers for the geographically parthenogenetic stick insect <i>Phraortes elongatus</i> (Insecta: Phasmatodea) Open
Many plant and animal species exhibit geographic parthenogenesis, wherein unisexual (= parthenogenetic) lineages are more common in their marginal habitats such as high latitude or altitudes than their closely related bisexual counterparts…
View article: SQSTM1L341V variant that is linked to sporadic ALS exhibits impaired association with MAP1LC3 in cultured cells
SQSTM1L341V variant that is linked to sporadic ALS exhibits impaired association with MAP1LC3 in cultured cells Open
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are genetically, pathologically and clinically-related progressive neurodegenerative diseases. Thus far, several SQSTM1 variations have been identified in patients with …
View article: Additional file 5: of Systemic overexpression of SQSTM1/p62 accelerates disease onset in a SOD1H46R-expressing ALS mouse model
Additional file 5: of Systemic overexpression of SQSTM1/p62 accelerates disease onset in a SOD1H46R-expressing ALS mouse model Open
Figure S5. Immunoblot-images used for the quantitative analysis in Fig. 6b. The immunoblots of (a) soluble misfolded SOD1 and GAPDH, (b) insoluble misfolded SOD1 and actin were analyzed for the quantitative analysis (Fig. 6b). The spinal c…
View article: Additional file 4: of Systemic overexpression of SQSTM1/p62 accelerates disease onset in a SOD1H46R-expressing ALS mouse model
Additional file 4: of Systemic overexpression of SQSTM1/p62 accelerates disease onset in a SOD1H46R-expressing ALS mouse model Open
Figure S4. Immunoblot-images used for the quantitative analysis in Figs. 6a and Fig. 7. The immunoblots of (a) soluble SOD1, (b) insoluble SOD1 monomer (Mono), (c) insoluble high-molecular weight (HMW) SOD1, (d) soluble SQSTM1, (e) insolu…
View article: Relationship Among Establishment Durations, Kin Relatedness, Aggressiveness, and Distance Between Populations of Eight Invasive Argentine Ant (Hymenoptera: Formicidae) Supercolonies in Japan
Relationship Among Establishment Durations, Kin Relatedness, Aggressiveness, and Distance Between Populations of Eight Invasive Argentine Ant (Hymenoptera: Formicidae) Supercolonies in Japan Open
We investigated kin relatedness and kin-recognition abilities of the Argentine ant, Linepithema humile (Mayr), an invader from North America that has pervaded Japan for 20 yr, using genetic analyses and behavioral bioassays. From these dat…
View article: Functional links between SQSTM1 and ALS2 in the pathogenesis of ALS: cumulative impact on the protection against mutant SOD1-mediated motor dysfunction in mice
Functional links between SQSTM1 and ALS2 in the pathogenesis of ALS: cumulative impact on the protection against mutant SOD1-mediated motor dysfunction in mice Open
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by a selective loss of motor neurons in the brain and spinal cord. Multiple toxicity pathways, such as oxidative stress, misfolded protein accumulation…