This report describes general characteristics of the group of diseases classified as amyotrophic lateral sclerosis (ALS). Amyotrophic lateral sclerosis is a genetically heterogeneous disorder, with multiple genes and mapped loci. A listing of ALS subtypes, as defined by OMIM, may be found in the table below, with links to more detailed reports for subtypes that have been investigated using fly models.
[updated May 2015 by FlyBase; FBrf0222196]
Amyotrophic lateral sclerosis is a neurodegenerative disorder characterized by the death of motor neurons in the brain, brainstem, and spinal cord, resulting in fatal paralysis. ALS usually begins with asymmetric involvement of the muscles in middle adult life. Approximately 10% of ALS cases are familial (Siddique and Deng, 1996, pubmed:8875253). ALS is sometimes referred to as 'Lou Gehrig disease' after the famous American baseball player who was diagnosed with the disorder. [from MIM:105400, 2015.02.11]
ALS is a genetically heterogeneous disorder, with several causative genes and mapped loci. [from MIM:105400, 2015.02.11]
A recent emerging theme in ALS research is the hypothesis that some ALS-associated proteins have a key role in the formation and function of cytoplasmic RNP stress granules, a cytosolic component in which non-functional translation initiation products accumulate. Stress granules form in response to a number of environmental stresses known to impede translation of mRNA into protein. Several ALS-associated proteins that have prion-like domains have been identified as accumulating in stress granules, including TARDBP, FUS, FUS-like proteins EWSR1 and TAF15, and HNRNPA1.
