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TDP43 was first identified as a novel cellular protein that binds to HIV-1 virus TAR DNA sequence motifs and acts a transcriptional repressor to the HIV-1 LTR. Later experiments revealed that TDP43 also regulates the splicing of exon 9 of the cystic fibrosis transmembrane conductance regular (CFTR), most likely through the association with the UG repeats at the 3"e; splice site. A hyperphosphorylated, ubiquitinated, and cleaved form of TDP43 known as pathologic TDP43 is the major disease protein in ubiquitin-positive, tau-, and alpha-synuclein-negative frontotemporal dementia (FLTD-U). TDP43 is not related to TRBP1, and RNA binding protein that binds HIV-1 TAR RNA sequences.
1190002A23Rik; ALS10; C85084; hypothetical protein; OTTHUMP00000002171; OTTHUMP00000002173; RCJMB04_5g9; RP4-635E18.2; TAR DNA binding protein; TAR DNA binding protein L homeolog; TAR DNA-binding protein 43; TAR DNA-binding protein-43; tardbp; tardbp.L; Tdp43; TDP-43; wu:fb77f02; wu:fc52g10; XELAEV_18035188mg
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