RediMODEL transgenics in C. elegans
TARDBP - TAR DNA-binding protein 43
- Function: TARDBP is transactive response DNA binding protein 43 kDa (TRD-43) and acts as a transactive repressor.
- Disease: Genetic defects in TARDBP have been found to be highly associated with Amyotrophic Lateral Sclerosis (ALS).
RediMODEL Kit - a series of humanized animal models specifically engineered for easy measurement of phenotype defects caused by clinically-relevant disease mutations.
TARDBP RediMODEL KO: Generated in the C. elegans homolog tdp-1 is a partial-deletion allele which leads to over 50% deletion in the coding sequence. The tdp-1 deletion knock-out (KO) results in loss of function in oxidative stress response. Major phenotype readout for KO line is the observation of hypersensitivity to oxidative stress. An outcrossed version of the partial-deletion mutant is currently available for preorder as gene knock-out.
TARDBP RediMODEL KI: Generated in the C. elegans homolog tdp-1 with human gene insertion to create a knock-in (KI) strain. Entire coding of tdp-1 is replaced with wild-type TARDBP coding sequence. A TARDBP gene replacement is currently available for preorder as a humanized wt line.
TARDBP RediMODEL CV: Generated in the C. elegans homolog tdp-1 with human gene insertion containing a clinical variant allele to create a clinical-variant (CV) strain. Entire coding of tdp-1 is replaced with TARDBP coding sequence containing amino-acid change polymorphism that has been observed in patient populations. Upon request, a TARDBP line with client-specific polymorphism is made as a clinical variant allele.
2. TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans, Parker et al., PLoS Genet. 2012 Jul;8(7):e1002806. doi: 10.1371/journal.pgen.1002806. Epub 2012 Jul 5.
3. Maple Syrup Decreases TDP-43 Proteotoxicity in a Caenorhabditis elegans Model of Amyotrophic Lateral Sclerosis (ALS), Therrien et al., J Agric Food Chem. 2016 May 4;64(17):3338-44. doi: 10.1021/acs.jafc.5b05432. Epub 2016 Apr 20.