Tar DNA Binding Protein 43 (TDP43) ELISA Kit for Rat

TARDBP: DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR. Homodimer. Binds specifically to pyrimidine-rich motifs of TAR DNA and to single stranded TG repeated sequences. Binds to RNA, specifically to UG repeated sequences with a minimun of six contiguous repeats. Interacts with ATNX2; the interaction is RNA-dependent. Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: RNA-binding

Chromosomal Location of Human Ortholog: 1p36.22

Cellular Component: nucleoplasm; perichromatin fibrils; cytoplasm; nuclear speck; nucleus

Molecular Function: identical protein binding; protein binding; mRNA 3'-UTR binding; RNA binding; double-stranded DNA binding; nucleotide binding; transcription factor activity

Biological Process: transcription from RNA polymerase II promoter; RNA splicing; positive regulation of insulin secretion; positive regulation of transcription from RNA polymerase II promoter; mRNA processing

Disease: Amyotrophic Lateral Sclerosis 10, With Or Without Frontotemporal Dementia