TDP43 Recombinant Rabbit Monoclonal Antibody [PSH09-14] (HA723072)

UNIPROT #

Q13148

PROTEIN NAME

TDP43

SYNONYMS

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

SUBCELLULAR LOCATION

Cytoplasm, Mitochondrion, Nucleus.

FUNCTION

TDP-43 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. TDP-43 has been shown to bind both DNA and RNA and have multiple functions in transcriptional repression, pre-mRNA splicing and translational regulation. Recent work has characterized the transcriptome-wide binding sites revealing that thousands of RNAs are bound by TDP-43 in neurons. TDP-43 was originally identified as a transcriptional repressor that binds to chromosomally integrated trans-activation response element (TAR) DNA and represses HIV-1 transcription. It was also reported to regulate alternate splicing of the CFTR gene and the apoA-II gene. In spinal motor neurons TDP-43 has also been shown in humans to be a low molecular weight neurofilament (hNFL) mRNA-binding protein. It has also shown to be a neuronal activity response factor in the dendrites of hippocampal neurons suggesting possible roles in regulating mRNA stability, transport and local translation in neurons. It has been demonstrated that zinc ions are able to induce aggregation of endogenous TDP-43 in cells. Moreover, zinc could bind to RNA binding domain of TDP-43 and induce the formation of amyloid-like aggregates in vitro.