PREY
DBR1
PRP26, L000000495, YKL149C
RNA lariat debranching enzyme; catalyzes debranching of lariat introns formed during pre-mRNA splicing; required for efficient Ty1 transposition; knockdown of human homolog Dbr1 rescues toxicity of RNA-binding proteins TDP-43 and FUS which are implicated in amyotrophic lateral sclerosis (ALS), suggests potential therapeutic target for ALS and related TDP-43 proteinopathies
GO Process (5)
GO Function (1)
GO Component (1)
Gene Ontology Biological Process
Gene Ontology Molecular Function
Saccharomyces cerevisiae (S288c)
Dosage Rescue
A genetic interaction is inferred when over expression or increased dosage of one gene rescues the lethality or growth defect of a strain that is mutated or deleted for another gene.
Publication
Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models.
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease primarily affecting motor neurons. Mutations in the gene encoding TDP-43 cause some forms of the disease, and cytoplasmic TDP-43 aggregates accumulate in degenerating neurons of most individuals with ALS. Thus, strategies aimed at targeting the toxicity of cytoplasmic TDP-43 aggregates may be effective. Here, we report results from two genome-wide loss-of-function ... [more]
Nat. Genet. Dec. 01, 2012; 44(12);1302-9 [Pubmed: 23104007]
Throughput
- Low Throughput
Ontology Terms
- phenotype: vegetative growth (APO:0000106)
Additional Notes
- Figure 1
Curated By
- BioGRID