Recombinant Human TAR DNA-binding protein 43 (TARDBP)

Uniprot Id

Q13148

Target Species

Human

Target Name

TARDBP

Target Full Name

TAR DNA-binding protein 43

Target Function

RNA-binding protein that is involved in various steps of RNA biogenesis and processing. 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. 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. Plays a role in maintaining mitochondrial homeostasis by regulating the processing of mitochondrial transcripts. Regulates also mRNA stability by recruiting CNOT7/CAF1 deadenylase on mRNA 3'UTR leading to poly(A) tail deadenylation and thus shortening. In response to oxidative insult, associates with stalled ribosomes localized to stress granules (SGs) and contributes to cell survival. Participates also in the normal skeletal muscle formation and regeneration, forming cytoplasmic myo-granules and binding mRNAs that encode sarcomeric proteins. Plays a role in the maintenance of the circadian clock periodicity via stabilization of the CRY1 and CRY2 proteins in a FBXL3-dependent manner. Negatively regulates the expression of CDK6. Regulates the expression of HDAC6, ATG7 and VCP in a PPIA/CYPA-dependent manner.

Target Involvement

Amyotrophic lateral sclerosis 10 (ALS10)

Target Subcellular Location

Nucleus. Cytoplasm. Cytoplasm, Stress granule.

Target Tissue Specificity

Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.

Target Research Area

Transcription, Microbiology

Target Synonyms

ALS10; OTTHUMP00000002171; OTTHUMP00000002172; OTTHUMP00000002173; TADBP_HUMAN; TAR DNA binding protein 43; TAR DNA binding protein; TAR DNA-binding protein 43; TARDBP; TDP 43; TDP-43; TDP43

Target Background

HIV-1, the causative agent of acquired immunodeficiency syndrome (AIDS), contains an RNA genome that produces a chromosomally integrated DNA during the replicative cycle. Activation of HIV-1 gene expression by the transactivator Tat is dependent on an RNA regulatory element (TAR) located downstream of the transcription initiation site. The protein encoded by this gene is a transcriptional repressor that binds to chromosomally integrated TAR DNA and represses HIV-1 transcription. In addition, this protein regulates alternate splicing of the CFTR gene. A similar pseudogene is present on chromosome 20.