Recombinant Mouse TAR DNA-binding protein 43 (Tardbp)

1. Findings highlight that the phosphatase regulator, GADD34, also functions as a kinase scaffold in response to chronic oxidative stress and recruits CK1 and oxidized TDP-43 to facilitate its phosphorylation, as seen in TDP-43 proteinopathies. PMID: 29109149
2. Increased excitatory synaptic inputs and dendritic spine densities is associated with TDP-43(Q331K) mutation resulting in amyotrophic lateral sclerosis. PMID: 27897242
3. Given the close association of stress granules and TDP-43, we wondered whether internalisation of SOD1 aggregates stimulated TDP-43 cytosolic aggregate structures. Addition of recombinant mutant G93A SOD1 aggregates to NSC-34 cells was found to trigger a rapid shift of TDP-43 to the cytoplasm where it was still accumulated after 48 h. PMID: 28560609
4. The mutated TDP-43 has a significant pathological effect at the dendritic spine that is associated with attenuated neural transmission. PMID: 27496536
5. Studied expression and localization of TAR DNA-binding protein 43 (TDP-43) in mouse seminiferous epithelium. Found TDP-43 is expressed by both germ cells and Sertoli cells and appears to have a role in specific stages of spermatogenesis. PMID: 28600885
6. TDP-43 overexpression decreases amyloid-Beta plaque deposition while increasing abnormal tau aggregation. PMID: 28416393
7. pTDP-43 granules were cleared and the number of pTDP-43-positive neurons returned to baseline after traumatic injury PMID: 28941811
8. results suggest that regulation of the U6 snRNA expression level by TDP-43 is a key factor in the increase in cell death upon TDP-43 loss-of-function PMID: 29125873
9. this study shows that HSF1 overexpression protects against TDP-43 pathology by upregulation of chaperones, especially HSP70, rather than enhancing autophagy PMID: 26994698
10. Acetylation of the protein triggers TDP-43 pathology in cultured cells and mouse skeletal muscle, which can be cleared through an HSF1-dependent chaperone mechanism that disaggregates the protein. PMID: 28724966
11. These studies showed that physiological oligomerization of TDP-43 is mediated through its N-terminal domain, which forms functional and dynamic oligomers antagonizing pathologic aggregation. PMID: 28663553
12. Study reports the altered expression and/or mislocalization of the TAR-DNA binding protein 43 (TDP-43) in both niemann-pick disease type C mouse and in a human neuronal model of the disease. Results extend the importance of the role of TDP-43 in neurodegenerative disease and further highlight the need to prioritize the targeting of this protein to develop novel therapeutic strategies. PMID: 27193329
13. superoxide dismutase function of SOD1 might not be required to preserve DNA integrity in motor neurons, at least when the function of TDP-43 is unaltered PMID: 28832631
14. The findings of this study support a role for nuclear depletion of TDP-43 in the pathogenesis of AD and provide strong rationale for developing novel therapeutics to alleviate the depletion of TDP-43 and functional antemortem biomarkers associated with its nuclear loss. PMID: 27785573
15. These results suggested that nuclear localization signal -tagged TDP25 (a carboxyl-terminal fragment of TDP-43) can change its structure to use ordered oligomeric but nontoxic state. Moreover, the structure of ordered oligomers as well as nuclear sequestration may be important in mediating cytotoxicity in ALS pathology. PMID: 28497562
16. Mutatgion M337V in TDP-43 impaired the Nrf2/ARE pathway by reducing the expression of MafK and JDP2 proteins. PMID: 28510254
17. The suppression of TDP-43 mitochondrial localization abolishes WT and mutant TDP-43-induced mitochondrial dysfunction and neuronal loss, and improves phenotypes of transgenic mutant TDP-43 mice. PMID: 27348499
18. These results establish that SMN overexpression in motor neurons slows disease onset and outcome by ameliorating pathological signs in this model of mutant TDP-43-mediated amyotrophic lateral sclerosis (ALS). PMID: 27466204
19. These findings suggest that TDP-43 promotes tau exon 10 inclusion and 4R-tau expression and that disease-related changes of TDP-43, truncations and mutations, affect its function in tau exon 10 splicing, possibly because of TDP-43 mislocalization to the cytoplasm. PMID: 28487370
20. this study suggests hemizygous TDP-43(M337V) mice as a useful animal model to study TDP-43 toxicity and further consolidates mitochondrial TDP-43 as a novel therapeutic target for TDP-43-linked neurodegenerative diseases. PMID: 28129109
21. demonstrated that the levels of HSF1 and heat shock proteins are significantly reduced in affected neuronal tissues from a TDP-43 transgenic mouse model of amyotrophic lateral sclerosis and patients with sporadic amyotrophic lateral sclerosis. PMID: 26936937
22. The present study identified USP7 and TDP-43 as the regulators of CRY1 and CRY2, underscoring the significance of the stability control process of CRY proteins for period determination in the mammalian circadian clockwork. PMID: 27123980
23. TDP-43 functions within a network of hnRNP proteins to inhibit the production of a truncated human SORT1 receptor. PMID: 26614389
24. Data demonstrate the existence of a physiological decrease of TDP-43/TBPH levels with aging in brain tissue both in wild-type mice and flies, showing that it is an evolutionary conserved phenomenon PMID: 26518462
25. This study demonistrated that proprioceptive nerve endings in muscles revealed early and alterations at Ia/II proprioceptive nerve endings in muscle spindles and in the absence of alterations in alpha-motor axons in TDP43(A315T) transgenic mice. PMID: 26136049
26. UBQLN2 dysregulation in neurons can drive NF-kappaB activation and cytosolic TDP-43 aggregation. PMID: 26521126
27. These findings demonstrate a role for PABPN1 in rescuing several cytopathological features of TDP-43 proteinopathy by increasing the turnover of pathologic proteins. PMID: 26130692
28. Valproate Attenuates 25-kDa C-Terminal Fragment of TDP-43-Induced Neuronal Toxicity via Suppressing Endoplasmic Reticulum Stress and Activating Autophagy. PMID: 26078717
29. we observed a glial reaction and an activity-dependent modification of Shh, Noggin, and Numb proteins. we found that Shh and Noggin could affect motor performance and that these proteins could be associated with both TDP-43 and Numb PMID: 26064939
30. The ability of TDP-43 to promote CD14-mediated activation of microglial NF-kappaB and AP-1 pathways, as well as the NLRP3 inflammasome. PMID: 26222336
31. Depletion of TDP-43 leads to a dramatic reduction in the RNA processing and the protein levels of IL-6 in serum. PMID: 25557830
32. our results reveal a previously unrecognized non-cell-autonomous mechanism in TDP-43-mediated neurodegeneration, identifying COX-2-PGE2 as the molecular events of microglia- but not astrocyte-initiated neurotoxicity PMID: 25811799
33. Results reveal a previously underappreciated complexity to TDP-43 regulated splicing and suggest that loss of TDP-43 autoregulatory capacity may contribute to the pathogenesis of amyotrophic lateral sclerosis. PMID: 26089325
34. Overexpression or depletion of TDP-43 in pre-adipose and adipose cells causes reciprocal alteration of IL-6 expression and RNA processing PMID: 26037142
35. NF-L protein or mRNA may negatively affect the expression of TDP-43 in the central nervous system. PMID: 25457553
36. Results indicate TDP-43 modifications (phosphorylation and truncation) increased and that modified TDP-43 was localized in astrocytes and microglial cells of the spinal cord in the hSOD1G93A amyotrophic lateral sclerosis mouse model PMID: 25213598
37. TDP-43 modulates the rCGG repeat-mediated neurodegeneration in a mouse model of fragile X tremor ataxia syndrome. PMID: 24986919
38. Gene targeting of mouse TDP-43 negatively affects Masp2 expression. PMID: 24740308
39. TDP-43 (A315T) mice suffer from intestinal dysmotility due to degeneration of NOS neurons in the myenteric plexus PMID: 24938805
40. These results indicate that TDP-43 mice display several core behavioral features of Frontotemporal dementia with motor neuron disease. PMID: 25392493
41. evidence for ER stress as a pathogenic pathway in TDP-43-mediated disease PMID: 24312274
42. TDP-43 regulates Ran expression, likely via binding to its 3'-UTR. PMID: 25155018
43. The Tardbp(Q101X) mutant mice are a useful tool for the dissection of TDP43 protein regulation, effects on splicing, embryonic development and neuromuscular phenotypes. PMID: 24465814
44. Defects of retrograde mitochondrial transport were detected at 45 days of age, before the onset of symptoms, in SOD1(G93A) and TDP43(A315T) mice, but not in SOD1(Wild Type). PMID: 24154542
45. This is the first evidence that TDP-43 might be involved in acute neuroinjury and TBI pathology PMID: 24917042
46. results suggest that TDP-43 dysfunction in astrocytes is an important driver for motor neuron degeneration and clinical phenotypes of ALS PMID: 24616503
47. TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis. PMID: 23967244
48. mTdp-43 downregulation does not lead to a loss of function mechanism or account for the pathological phenotypes observed in hTDP-43 homozygous mice. PMID: 23922830
49. Accumulation of the transactive response DNA-binding protein 43 (TDP-43) is a major hallmark of several neurodegenerative disorders, collectively known as TDP-43 proteinopathies. PMID: 23954172
50. Exogenous progestorone (9300-93,000 pg/ml) had a significant effect on overall Tau and nuclear TDP-43 levels, reducing total Tau levels by approximately 1.5-fold and increasing nuclear TDP-43 by 1.7- to 2.0-fold. PMID: 23798570

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KEGG: mmu:230908

STRING: 10090.ENSMUSP00000081142

UniGene: Mm.22453