Anti-TDP43 / TARDBP / TAR DNA binding protein, clone TARDP/349

1 mg/ml in 1X PBS, BSA free, sodium azide free. TARDBP (TAR DNA binding protein), also known as TDP-43, is a nuclear protein that contains two RRM (RNA recognition motif) domains. Ubiquitously expressed with highest levels found in placenta, lung, pancreas, spleen and genital tract, TARDBP functions as a DNA-binding protein and specifically binds to the TAR DNA sequence motifs of HIV. Via this association with TAR motifs, TARDBP acts as a transcriptional repressor and inhibits HIV-1 transcription. TARDBP can also function as a negative regulator of splicing activity and is known to be involved in the splicing of CFTR (cystic fibrosis transmembrane receptor). In addition, TARDBP is a major component of ubiquitin-positive inclusion bodies that are prominent in many neurodegenerative diseases. This suggests that TARDBP may play a role in the development of neurodegenerative disorders. Due to alternative splicing events, various isoforms exist for TARDBP. Protein function: RNA-binding protein that is involved in various steps of RNA biogenesis and processing (PubMed:23519609). Preferentially binds, via its two RNA recognition motifs RRM1 and RRM2, to GU-repeats on RNA molecules predominantly localized within long introns and in the 3'UTR of mRNAs (PubMed:23519609, PubMed:24240615, PubMed:24464995). In turn, regulates the splicing of many non-coding and protein-coding RNAs including proteins involved in neuronal survival, as well as mRNAs that encode proteins relevant for neurodegenerative diseases (PubMed:21358640, PubMed:29438978). Plays a role in maintaining mitochondrial homeostasis by regulating the processing of mitochondrial transcripts (PubMed:28794432). Regulates also mRNA stability by recruiting CNOT7/CAF1 deadenylase on mRNA 3'UTR leading to poly(A) tail deadenylation and thus shortening (PubMed:30520513). In response to oxidative insult, associates with stalled ribosomes localized to stress granules (SGs) and contributes to cell survival (PubMed:23398327, PubMed:19765185). Participates also in the normal skeletal muscle formation and regeneration, forming cytoplasmic myo-granules and binding mRNAs that encode sarcomeric proteins (PubMed:30464263). Plays a role in the maintenance of the circadian clock periodicity via stabilization of the CRY1 and CRY2 proteins in a FBXL3-dependent manner (PubMed:27123980). Negatively regulates the expression of CDK6 (PubMed:19760257). Regulates the expression of HDAC6, ATG7 and VCP in a PPIA/CYPA-dependent manner (PubMed:25678563). [The UniProt Consortium]