TAR DNA-binding protein 43 (TDP-43) is an RNA binding protein (RBP) that has been shown to bind both DNA and RNA and have multiple functions in transcriptional repression, pre-mRNA splicing and translational regulation. It belongs to the hnRNP protein family and is highly expressed in the pancreas, placenta, lung, genital tract and spleen[1]. Characterisation of transcriptome-wide binding sites revealed that thousands of RNAs are bound by TDP-43 in neurons.
Mutations in TDP-43 have been associated with amyotrophic lateral sclerosis, frontotemporal dementia, Parkinson's disease and Alzheimer's disease.
TDP-43 is predominantly located in the nucleus under normal physiological conditions. However, hyperphosphorylated, fragmented and ubiquitinated forms of TDP-43 have been identified as core components of cytosolic inclusions in sporadic ALS and frontotemporal lobar degeneration (FTLD) [2,3,4,5,6,7,8].
TDP-43 contains a nuclear localising signal (NLS) as well as a nuclear export signal (NES)[8], which enables the shuttling of TDP-43 between the nucleus and the cytosol. Under normal conditions, TDP-43 interacts with mRNAs on which ribosomes are located separately, forming polysomes. Various stresses can induce clustering of ribosomes into a stalled state, resulting in the formation of stress granules (SG) containing TIA-1, G3BP, ataxin-2 and eIF4G1/2.
In the stalled state, transcription is inhibited in a homeostatic response. However, sustained stress and TDP-43 misfolding creates aberrant SGs and pathogenic TDP-43 aggregates [9]. Misfolding and cytosolic mislocalisation also lead directly to a loss of normal TDP-43 function, and the resultant disruption of protein and RNA homeostasis is considered another likely pathogenic mechanism in addition to the toxicity of inclusions in ALS[9].
Product Type:
NS Reagents Antibody
Antibody Type:
Monoclonal
Format:
100 µg in 100 µl PBS containing 0.02% sodium azide.
If you would like us to check if this antibody is likely to bind to this protein from a different species please contact us. We are happy to check for you.
Immunogen:
A His-tagged recombinant protein from the C-termnal of human TDP-43 (aa 208-414)
If you would like further information regarding the immunogen used in the production of this antibody or have a query about whether this antibody will bind to your protein/species please contact us and we can do the analysis for you.
Clone number:
Clone DB9
Antibody Isotype:
IgG1 kappa
Application Details:
ELISA, IHC 1:200, WB 1:1000, ICC 1:200
Antibody Number:
10010
Category:
Primary Antibodies
Other names:
TAR DNA-binding protein 43, TDP-43, transactive response DNA binding protein 43 kDa
43kDa (Intended as a general guide and does not allow for all isoforms and species variations)
Subcellular location:
Nucleus
Purification:
Protein A Affinity Purified
References:
1. Strong MJ, Volkening K, Hammond R, et al. (2007). TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein. Molecular and Cellular Neuroscience. 35 (2): 3207.
2. Kwong LK, Neumann M, Sampathu DM, et al. (2007). TDP-43 proteinopathy: The neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. Acta Neuropathologica. 114 (1): 6370.
3. Arai, T. et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem. Biophys. Res. Commun. 351, 602611 (2006). CAS
4. Neumann M, Sampathu DM, Kwong LK, Truax AC, et al. (2006). Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis. Science. 314 (5796): 1303.
5. Tan, C. F. et al. TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation. Acta Neuropathol. 113, 535542 (2007).
6. Igaz, L. M. et al. Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Am. J. Pathol. 173, 182194 (2008).
7. Tremblay C, St-Amour I, Schneider J, et al. (2011). Accumulation of transactive response DNA binding protein 43 in mild cognitive impairment and Alzheimer disease. J Neuropathol Exp Neurol. 70 (9): 78898.
8. Winton, M. J. et al. Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation. J. Biol. Chem. 283, 1330213309 (2008).
9. Yoshitaka Tamaki et al. Elimination of TDP-43 inclusions linked to amyotrophic lateral sclerosis by a misfolding-specific intrabody with dual proteolytic signals. Scientific Reports: volume 8, Article number: 6030 (2018)
TAR DNA-binding protein 43 (TDP-43) is an RNA binding protein (RBP) that has been shown to bind both DNA and RNA and have multiple functions in transcriptional repression, pre-mRNA splicing and translational regulation. It belongs to the hnRNP protein family and is highly expressed in the pancreas, placenta, lung, genital tract and spleen[1]. Characterisation of transcriptome-wide binding sites revealed that thousands of RNAs are bound by TDP-43 in neurons.
Mutations in TDP-43 have been associated with amyotrophic lateral sclerosis, frontotemporal dementia, Parkinson's disease and Alzheimer's disease.
TDP-43 is predominantly located in the nucleus under normal physiological conditions. However, hyperphosphorylated, fragmented and ubiquitinated forms of TDP-43 have been identified as core components of cytosolic inclusions in sporadic ALS and frontotemporal lobar degeneration (FTLD) [2,3,4,5,6,7,8].
TDP-43 contains a nuclear localising signal (NLS) as well as a nuclear export signal (NES)[8], which enables the shuttling of TDP-43 between the nucleus and the cytosol. Under normal conditions, TDP-43 interacts with mRNAs on which ribosomes are located separately, forming polysomes. Various stresses can induce clustering of ribosomes into a stalled state, resulting in the formation of stress granules (SG) containing TIA-1, G3BP, ataxin-2 and eIF4G1/2.
In the stalled state, transcription is inhibited in a homeostatic response. However, sustained stress and TDP-43 misfolding creates aberrant SGs and pathogenic TDP-43 aggregates [9]. Misfolding and cytosolic mislocalisation also lead directly to a loss of normal TDP-43 function, and the resultant disruption of protein and RNA homeostasis is considered another likely pathogenic mechanism in addition to the toxicity of inclusions in ALS[9].
Product Type:
NS Reagents Antibody
Antibody Type:
Polyclonal
Format:
100 µg in 100 µl PBS containing 0.02% sodium azide.
If you would like us to check if this antibody is likely to bind to this protein from a different species please contact us. We are happy to check for you.
Immunogen:
Partial length recombinant human TDP-43 from the N-terminal region of the protein
If you would like further information regarding the immunogen used in the production of this antibody or have a query about whether this antibody will bind to your protein/species please contact us and we can do the analysis for you.
Antibody Isotype:
IgG
Application Details:
WB 1:500-2000. IHC 1:50-100. IF 1:20-100. IP 1:50-100. RIP 1:20-50.
Antibody Number:
10049
Category:
Primary Antibodies
Other names:
TAR DNA-binding protein 43, TARDBP, transactive response DNA binding protein 43 kDa, ALS10
43kDa (Intended as a general guide and does not allow for all isoforms and species variations)
Subcellular location:
Nucleus
Purification:
Affinity purification
References:
1. Strong MJ, Volkening K, Hammond R, et al. (2007). TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein. Molecular and Cellular Neuroscience. 35 (2): 3207.
2. Kwong LK, Neumann M, Sampathu DM, et al. (2007). TDP-43 proteinopathy: The neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. Acta Neuropathologica. 114 (1): 6370.
3. Arai, T. et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem. Biophys. Res. Commun. 351, 602611 (2006). CAS
4. Neumann M, Sampathu DM, Kwong LK, Truax AC, et al. (2006). Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis. Science. 314 (5796): 1303.
5. Tan, C. F. et al. TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation. Acta Neuropathol. 113, 535542 (2007).
6. Igaz, L. M. et al. Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Am. J. Pathol. 173, 182194 (2008).
7. Tremblay C, St-Amour I, Schneider J, et al. (2011). Accumulation of transactive response DNA binding protein 43 in mild cognitive impairment and Alzheimer disease. J Neuropathol Exp Neurol. 70 (9): 78898.
8. Winton, M. J. et al. Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation. J. Biol. Chem. 283, 1330213309 (2008).
9. Yoshitaka Tamaki et al. Elimination of TDP-43 inclusions linked to amyotrophic lateral sclerosis by a misfolding-specific intrabody with dual proteolytic signals. Scientific Reports: volume 8, Article number: 6030 (2018)
Western Blotting (WB), Immunocytochemistry (ICC) and Immunohistochemistry (IHC). A dilution of 1:1,000 - 1:5,000 is recommended for WB and IHC. A dilution of 1:500-1,000 is recommended for IC. Biosensis recommends optimal dilutions/concentrations should be determined by the end user.
Alternative Names:
TAR DNA-binding protein 43; TDP-43; TARDBP; TDP43;
Accession Number:
Q13148 TADBP_HUMAN;
Reconstitution:
Reconstitute in sterile distilled water. Centrifuge to remove any insoluble material.
Shelf Life:
12 months after purchase
Specificity:
The specificity of this antibody has been confirmed by WB. This antibody detects ~43 kDa TDP43 protein on crude extract of mouse brain nuclear fraction. | Human and Rodent. Predicted to react with other mammalian tissue.
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
Phospho-TDP43 ( Ser409) Antibody detects endogenous levels of TDP43 only when phosphorylated at Ser409
Epitope:
Phospho Ser409
Immunogen:
A synthesized peptide derived from human TDP43 around the phosphorylation site of Ser409
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antibody is from purified rabbit serum by affinity purification via sequential chromatography on phospho- and non-phospho-peptide affinity columns.
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
TDP43 Antibody detects endogenous levels of total TDP43
Immunogen:
A synthesized peptide derived from human TDP43
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
TARDBP Antibody detects endogenous levels of total TARDBP
Immunogen:
A synthesized peptide derived from human TARDBP
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
Phospho-TDP43 ( Ser409) Antibody detects endogenous levels of TDP43 only when phosphorylated at Ser409
Epitope:
Phospho Ser409
Immunogen:
A synthesized peptide derived from human TDP43 around the phosphorylation site of Ser409
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antibody is from purified rabbit serum by affinity purification via sequential chromatography on phospho- and non-phospho-peptide affinity columns.
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
TDP43 Antibody detects endogenous levels of total TDP43
Immunogen:
A synthesized peptide derived from human TDP43
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
TARDBP Antibody detects endogenous levels of total TARDBP
Immunogen:
A synthesized peptide derived from human TARDBP
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
TAR DNA-binding protein 43 (TDP-43) is an RNA binding protein (RBP) that has been shown to bind both DNA and RNA and have multiple functions in transcriptional repression, pre-mRNA splicing and translational regulation. It belongs to the hnRNP protein family and is highly expressed in the pancreas, placenta, lung, genital tract and spleen[1]. Characterisation of transcriptome-wide binding sites revealed that thousands of RNAs are bound by TDP-43 in neurons.
Mutations in TDP-43 have been associated with amyotrophic lateral sclerosis, frontotemporal dementia, Parkinson's disease and Alzheimer's disease.
TDP-43 is predominantly located in the nucleus under normal physiological conditions. However, hyperphosphorylated, fragmented and ubiquitinated forms of TDP-43 have been identified as core components of cytosolic inclusions in sporadic ALS and frontotemporal lobar degeneration (FTLD) [2,3,4,5,6,7,8].
TDP-43 contains a nuclear localising signal (NLS) as well as a nuclear export signal (NES)[8], which enables the shuttling of TDP-43 between the nucleus and the cytosol. Under normal conditions, TDP-43 interacts with mRNAs on which ribosomes are located separately, forming polysomes. Various stresses can induce clustering of ribosomes into a stalled state, resulting in the formation of stress granules (SG) containing TIA-1, G3BP, ataxin-2 and eIF4G1/2.
In the stalled state, transcription is inhibited in a homeostatic response. However, sustained stress and TDP-43 misfolding creates aberrant SGs and pathogenic TDP-43 aggregates [9]. Misfolding and cytosolic mislocalisation also lead directly to a loss of normal TDP-43 function, and the resultant disruption of protein and RNA homeostasis is considered another likely pathogenic mechanism in addition to the toxicity of inclusions in ALS[9].
If you would like us to check if this antibody is likely to bind to this protein from a different species please contact us. We are happy to check for you.
Immunogen:
A His-tagged recombinant protein from the C-termnal of human TDP-43 (aa 208-414)
If you would like further information regarding the immunogen used in the production of this antibody or have a query about whether this antibody will bind to your protein/species please contact us and we can do the analysis for you.
Clone number:
Clone DB9
Antibody Isotype:
IgG1 kappa
Application Details:
ELISA, IHC 1:200, WB 1:1000, ICC 1:200
Antibody Number:
10010
Category:
Primary Antibodies
Other names:
TAR DNA-binding protein 43, TDP-43, transactive response DNA binding protein 43 kDa. ALS10
43kDa (Intended as a general guide and does not allow for all isoforms and species variations)
Subcellular location:
Nucleus
Purification:
Protein A Affinity Purified
References:
1. Strong MJ, Volkening K, Hammond R, et al. (2007). TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein. Molecular and Cellular Neuroscience. 35 (2): 3207.
2. Kwong LK, Neumann M, Sampathu DM, et al. (2007). TDP-43 proteinopathy: The neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. Acta Neuropathologica. 114 (1): 6370.
3. Arai, T. et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem. Biophys. Res. Commun. 351, 602611 (2006). CAS
4. Neumann M, Sampathu DM, Kwong LK, Truax AC, et al. (2006). Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis. Science. 314 (5796): 1303.
5. Tan, C. F. et al. TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation. Acta Neuropathol. 113, 535542 (2007).
6. Igaz, L. M. et al. Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Am. J. Pathol. 173, 182194 (2008).
7. Tremblay C, St-Amour I, Schneider J, et al. (2011). Accumulation of transactive response DNA binding protein 43 in mild cognitive impairment and Alzheimer disease. J Neuropathol Exp Neurol. 70 (9): 78898.
8. Winton, M. J. et al. Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation. J. Biol. Chem. 283, 1330213309 (2008).
9. Yoshitaka Tamaki et al. Elimination of TDP-43 inclusions linked to amyotrophic lateral sclerosis by a misfolding-specific intrabody with dual proteolytic signals. Scientific Reports: volume 8, Article number: 6030 (2018)
TAR DNA-binding protein 43 (TDP-43) is an RNA binding protein (RBP) that has been shown to bind both DNA and RNA and have multiple functions in transcriptional repression, pre-mRNA splicing and translational regulation. It belongs to the hnRNP protein family and is highly expressed in the pancreas, placenta, lung, genital tract and spleen[1]. Characterisation of transcriptome-wide binding sites revealed that thousands of RNAs are bound by TDP-43 in neurons.
Mutations in TDP-43 have been associated with amyotrophic lateral sclerosis, frontotemporal dementia, Parkinson's disease and Alzheimer's disease.
TDP-43 is predominantly located in the nucleus under normal physiological conditions. However, hyperphosphorylated, fragmented and ubiquitinated forms of TDP-43 have been identified as core components of cytosolic inclusions in sporadic ALS and frontotemporal lobar degeneration (FTLD) [2,3,4,5,6,7,8].
TDP-43 contains a nuclear localising signal (NLS) as well as a nuclear export signal (NES)[8], which enables the shuttling of TDP-43 between the nucleus and the cytosol. Under normal conditions, TDP-43 interacts with mRNAs on which ribosomes are located separately, forming polysomes. Various stresses can induce clustering of ribosomes into a stalled state, resulting in the formation of stress granules (SG) containing TIA-1, G3BP, ataxin-2 and eIF4G1/2.
In the stalled state, transcription is inhibited in a homeostatic response. However, sustained stress and TDP-43 misfolding creates aberrant SGs and pathogenic TDP-43 aggregates [9]. Misfolding and cytosolic mislocalisation also lead directly to a loss of normal TDP-43 function, and the resultant disruption of protein and RNA homeostasis is considered another likely pathogenic mechanism in addition to the toxicity of inclusions in ALS[9].
If you would like us to check if this antibody is likely to bind to this protein from a different species please contact us. We are happy to check for you.
Immunogen:
Partial length recombinant human TDP-43 from the N-terminal region of the protein
If you would like further information regarding the immunogen used in the production of this antibody or have a query about whether this antibody will bind to your protein/species please contact us and we can do the analysis for you.
Antibody Isotype:
IgG
Application Details:
WB 1:500-2000. IHC 1:50-100. IF 1:20-100. IP 1:50-100. RIP 1:20-50.
Antibody Number:
10049
Category:
Primary Antibodies
Other names:
TAR DNA-binding protein 43, TARDBP, transactive response DNA binding protein 43 kDa, ALS10
43kDa (Intended as a general guide and does not allow for all isoforms and species variations)
Subcellular location:
Nucleus
Purification:
Affinity purification
References:
1. Strong MJ, Volkening K, Hammond R, et al. (2007). TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein. Molecular and Cellular Neuroscience. 35 (2): 3207.
2. Kwong LK, Neumann M, Sampathu DM, et al. (2007). TDP-43 proteinopathy: The neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease. Acta Neuropathologica. 114 (1): 6370.
3. Arai, T. et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem. Biophys. Res. Commun. 351, 602611 (2006). CAS
4. Neumann M, Sampathu DM, Kwong LK, Truax AC, et al. (2006). Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis. Science. 314 (5796): 1303.
5. Tan, C. F. et al. TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation. Acta Neuropathol. 113, 535542 (2007).
6. Igaz, L. M. et al. Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Am. J. Pathol. 173, 182194 (2008).
7. Tremblay C, St-Amour I, Schneider J, et al. (2011). Accumulation of transactive response DNA binding protein 43 in mild cognitive impairment and Alzheimer disease. J Neuropathol Exp Neurol. 70 (9): 78898.
8. Winton, M. J. et al. Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation. J. Biol. Chem. 283, 1330213309 (2008).
9. Yoshitaka Tamaki et al. Elimination of TDP-43 inclusions linked to amyotrophic lateral sclerosis by a misfolding-specific intrabody with dual proteolytic signals. Scientific Reports: volume 8, Article number: 6030 (2018)
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
Phospho-TDP43 ( Ser409) Antibody detects endogenous levels of TDP43 only when phosphorylated at Ser409
Epitope:
Phospho Ser409
Immunogen:
A synthesized peptide derived from human TDP43 around the phosphorylation site of Ser409
Cross Reactivity:
Human,Mouse,Rat
Conjugate:
Unconjugated
purification:
The antibody is from purified rabbit serum by affinity purification via sequential chromatography on phospho- and non-phospho-peptide affinity columns.
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.
The antiserum was purified by peptide affinity chromatography using SulfoLink(TM) Coupling Resin (Thermo Fisher Scientific).
Concentration:
1mg/ml
Buffer:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt
Gene ID:
23435
Gene:
TARDBP
Swiss-Prot:
Q13148
Molecular Weight:
43kDa
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