This antibody recognizes the cleavage product of 20-30 kDa in addition to the native and phosphorylated forms of TDP-43. Immunohistochemical analyses of TDP-43 using this antibody detect both normal diffuse nuclear staining and insoluble inclusions in pathologic tissues. Various forms of TDP-43 exist, including 18-35 kDa of cleaved C-terminal fragments, 45-50 kDa phosphoprotein, 55 kDa glycosylated form, 75 kDa hyperphosphorylated form, and 90-300 kDa cross-linked form.
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.
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Protein Aliases: OTTHUMP00000002171; OTTHUMP00000002173; RP4-635E18.2; TAR DNA-binding protein 43; TAR DNA-binding protein-43; TDP-43
Gene Aliases: 1190002A23Rik; ALS10; C85084; TARDBP; TDP-43; TDP43