TAR DNA-binding protein

Many crystal structures have been reported involve nucleic acid interactions with DNA and RNA polymerases as well as other nucleic acid replicative anzymes. " Other structures involve tRNA and tRNA synthetases, and many nucleic acid-modifying enzymes including methyl transferases, " " repair enzymes, " endonucleases, the RNase R enzyme Rrp44 and topoisomerases and helicases. Other protein-DNA structures reported include Soxl7 bound to DNA, " the Mosl paired-end complex, TAR DNA-binding protein (TDP-43) and the tumor suppressor protein p53. A number of protein-RNA structures are also reported including pseudouridine synthase, the E.coli SeqA... 

Olive M, Janue A, Moreno D et al. (2009) TAR DNA-Binding protein 43 accumulation in protein aggregate myopathies. J Neuropathol Exp Neurol 68, 262-21 i. 

Chen-Plotkin AS, Lee VM, Trojanowski JQ. (2010) TAR DNA-binding protein 43 in neurodegenerative disease. Nat Rev Neurol 6, 211-220. 

The myopathic features of IBMPFD vary tremendously between patients, but usually encompass the typical characteristics of inclusion-body myopathy (IBM), including the presence of ubiquitin-positive inclusions, centralized nuclei, and an increased prevalence of endomysial connective tissue [3, 10, 13]. In addition, inclusions containing TAR DNA-binding protein-43 (TDP-43), which colocahze with ubiquitin, are often found in the sarcolemma and sarcoplasm of affected musdes [14, 15]. However, TDP-43-positive inclusions are also found in several other myopathies, as well as dominantly inherited and sporadic cases of ALS [16], Other myopathic characteristics of IBMPFD may indude regional variations in muscle-fiber size, as weU as the presence of rimmed vacuoles and/or inflammatory infiltrates [3, 10, 11]. 

---