Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by the selective loss of motor neurons, ultimately leading to muscle weakness, paralysis, and death. Although ~85-90% of ALS cases are sporadic, mutations in over 40 genes have been identified that contribute to the development of ALS. Despite extensive research, the molecular mechanisms driving ALS onset and
\nprogression remain only partially understood, limiting the development of effective therapeutics. A major challenge in ALS research is the lack of physiologically relevant human models capable of capturing patient specific phenotypes at scale.<\/p>\n
Human induced pluripotent stem cell (iPSC) technology provides a powerful platform for generating disease relevant cell types directly from patient donors, including functional motor neurons. These cells retain the genetic background of the donor and can recapitulate ALS associated electrophysiological and morphological characteristics. Human iPSC-derived motor neurons from donors that carry the C9orf72 repeat expansion, the most common genetic cause of familial ALS, exhibit measurable alterations in ion channel function, excitability, and network behavior. Axol Bioscience manufactures high\u2011quality, functionally validated motor neurons representing key ALS\u2011associated genetic backgrounds, including C9orf72, SOD1, and TDP\u201143 mutations, providing robust, disease\u2011relevant cells for electrophysiological studies.<\/p>\n","protected":false},"featured_media":0,"template":"","products_tags":[42],"resource_type":[90],"application_areas":[50,53,77],"therapeutic_areas":[55,143],"target_categories":[61,337],"target_families":[],"targets":[],"resource_keyword":[],"class_list":["post-18928","resource_library","type-resource_library","status-publish","hentry","products_tags-syncropatch-384","resource_type-application-note","application_areas-ion-channels","application_areas-assay-development-and-validation","application_areas-cell-ipscs-profiling","therapeutic_areas-cns","therapeutic_areas-assay-development-and-validation","target_categories-voltage-gated-ion-channels","target_categories-ligand-gated-ion-channels"],"acf":[],"_links":{"self":[{"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/resource_library\/18928","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/resource_library"}],"about":[{"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/types\/resource_library"}],"wp:attachment":[{"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/media?parent=18928"}],"wp:term":[{"taxonomy":"products_tags","embeddable":true,"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/products_tags?post=18928"},{"taxonomy":"resource_type","embeddable":true,"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/resource_type?post=18928"},{"taxonomy":"application_areas","embeddable":true,"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/application_areas?post=18928"},{"taxonomy":"therapeutic_areas","embeddable":true,"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/therapeutic_areas?post=18928"},{"taxonomy":"target_categories","embeddable":true,"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/target_categories?post=18928"},{"taxonomy":"target_families","embeddable":true,"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/target_families?post=18928"},{"taxonomy":"targets","embeddable":true,"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/targets?post=18928"},{"taxonomy":"resource_keyword","embeddable":true,"href":"https:\/\/nanion.com\/ja\/wp-json\/wp\/v2\/resource_keyword?post=18928"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}