Implications of the prion-related Q/N domains in TDP-43 and FUS
نویسندگان
چکیده
منابع مشابه
Implications of the prion-related Q/N domains in TDP-43 and FUS.
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are clinically overlapping neurodegenerative disorders whose pathophysiology remains incompletely understood. ALS initiates in a discrete location, and typically progresses in a pattern consistent with spread of the degenerative process to involve neighboring regions of the motor system, although the basis of the a...
متن کاملRethinking ALS: The FUS about TDP-43
Mutations in TDP-43, a DNA/RNA-binding protein, cause an inherited form of the neurodegenerative disease amyotrophic lateral sclerosis (ALS). Two recent studies (Kwiatkowski et al., 2009; Vance et al., 2009) now report that mutations in FUS/TLS, another DNA/RNA-binding protein, also trigger premature degeneration of motor neurons. TDP-43 and FUS/TLS have striking structural and functional simil...
متن کاملA Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.
Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder characterized by the loss of motor neurons. Fused in sarcoma/translated in liposarcoma (FUS/TLS) and TAR DNA-binding protein (TDP)-43 are DNA/RNA-binding proteins found to be mutated in sporadic and familial forms of ALS. Ectopic expression of human ALS-causing FUS/TLS mutations in Drosophila caused an accumulation o...
متن کاملThe Functions of Glycine‐rich Regions in Tdp‐43, Fus and Related Rna‐binding Proteins
Glycine‐rich regions form intrinsically unstructured domains within RNA‐binding proteins. Although they lack a defined structure when alone in solution, these domains can form more defined structural elements when interacting with other proteins or with RNA. TDP‐43 and FUS are RNA binding proteins with glycine‐rich domains that form abnormal aggregates in amyotrophic lateral sclerosis (ALS) and...
متن کاملALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.
Dominant mutations in two functionally related DNA/RNA-binding proteins, trans-activating response region (TAR) DNA-binding protein with a molecular mass of 43 KDa (TDP-43) and fused in sarcoma/translocation in liposarcoma (FUS/TLS), cause an inherited form of ALS that is accompanied by nuclear and cytoplasmic aggregates containing TDP-43 or FUS/TLS. Using isogenic cell lines expressing wild-ty...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Prion
سال: 2011
ISSN: 1933-6896,1933-690X
DOI: 10.4161/pri.5.1.14265