Mouse Torsin A Monoclonal Antibody | anti-TOR1A antibody
Torsin A (Torsin family 1 member A, Dystonia 1 protein)
Ascites
Ascites
Dilution: Immunocytochemistry: 1:100 on 4% paraformaldehyde fixed (37 degree C for 30 minutes) cells. Suggested permeabilization method is 0.1% NP40 for 20 minutes.
Immunoprecipitation: The preferred tissue/cell lysis buffer is 1% NP40 and/or RIPA. Recommended antibody amount is 3-4ul of ascites. Final reaction volume of 1000ul. Final total protein concentration in reaction mix: 0.1-0.5mg/ml. Suggested incubation time is 16 hours. Sugggested capture is Protein A/G.
Western Blot: 1:200. Western blot: 1:200. The antibody reacts with the ~37 kDa torsinA protein. The suggested blocking buffer is TBS with 10% milk and 0.05% Tween-20. The suggested dilution buffer is TBS with 0.05% Tween-20. Recognizes Torsin A at ~3 7kD.
Ozelius et al. (1997) constructed a cosmid contig spanning the region of chromosome 9 to which early-onset torsion dystonia (128100) had been mapped. Using exon trapping and identification of cDNAs encoded in this region, and mutational screening by SSCP and sequence analysis of cDNA and genomic DNA from affected individuals and controls, they identified the DYT1 gene. The deduced protein, termed 'torsinA' by them, comprises 332 amino acids and has a calculated molecular mass of 37.813kD. It contains an ATP-binding domain and a putative N-terminal leader sequence. DYT1 has high homology to 3 additional mammalian genes and a nematode gene and distal similarity to the family of heat-shock proteins and the Clp protease family. Northern blot analysis detected 2 ubiquitously expressed transcripts of 1.8kb and 2.2kb and an additional low-abundance transcript of 5kb in fetal brain, lung, and kidney, as well as in adult brain, heart, and pancreas.
Using an mRNA probe for DYT1 in normal human postmortem brains, Augood et al. (1998) found high expression in melanized neurons of the pars compacta of the substantia nigra, as well as in the cerebellum and the dentate gyrus and stratum pyramidal of CA3.
Using polyclonal antibodies directed against human torsin-A and torsin-B, Konakova et al. (2001) analyzed the expression of the proteins in normal human brain regions and found widespread neuronal distribution. Areas of intense labeling included the dentate nucleus and Purkinje cells of the cerebellum, all layers of the cerebral cortex, all subfields of the hippocampus, particularly CA3, the thalamus, the spinal cord, and the midbrain, and there was weak labeling in the substantia nigra. Staining was predominantly cytoplasmic, but also extended into the axons, dendrites, and neuropil. Glial cells showed no expression of the proteins. Torsin B showed a similar pattern of expression, but was found to have some polarization toward the cell edge, and was detected in the pigmented cells of the substantia nigra. Both proteins were present at high levels in regions not affected by disease (cortex, cerebellum, spinal cord). Konakova et al. (2001) noted that the staining pattern was granular and present in the neuronal processes, suggesting a role in regulating neurotransmitter release.
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene is a member of the AAA family of adenosine triphosphatases (ATPases), is related to the Clp protease/heat shock family and is expressed prominently in the substantia nigra pars compacta. Mutations in this gene result in the autosomal dominant disorder, torsion dystonia 1. [provided by RefSeq, Jul 2008]
Uniprot Description
TOR1A: May serve as a molecular chaperone assisting in the proper folding of secreted and/or membrane proteins. In the nucleus, displaces the nuclear membrane proteins SUN2, SYNE2 and nesprin-3/C14orf49, leaving nuclear pores and SUN1 unchanged. May form homohexamers. Interacts with TOR1AIP1 and TOR1AIP2. Interacts with KLHL14, preferentially when ATP-free. Widely expressed. Highest levels in kidney and liver. Not detected in spleen. In the brain, high levels found in the dopaminergic neurons of the substantia nigra pars compacta, as well as in the neocortex, hippocampus and cerebellum. Also high expression in the spinal cord. Belongs to the clpA/clpB family. Torsin subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 9q34
Cellular Component: synaptic vesicle; transport vesicle; nuclear membrane; cytoskeleton; cytoplasmic vesicle membrane; growth cone; membrane; intracellular membrane-bound organelle; endoplasmic reticulum lumen; nuclear envelope; cell junction; secretory granule
Molecular Function: protein binding; kinesin binding; ATPase activity; cytoskeletal protein binding; unfolded protein binding; misfolded protein binding; ATP binding
Biological Process: protein deneddylation; chaperone cofactor-dependent protein folding; organelle organization and biogenesis; synaptic vesicle transport; regulation of dopamine uptake; intermediate filament cytoskeleton organization and biogenesis; response to oxidative stress; cell adhesion; neurite development; protein homooligomerization; nuclear membrane organization and biogenesis
Disease: Dystonia 1, Torsion, Autosomal Dominant
Research Articles on TOR1A
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Product Notes
The TOR1A tor1a (Catalog #AAA604063) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Torsin A (Torsin family 1 member A, Dystonia 1 protein) reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Torsin A can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunoprecipitation (IP), Immunocytochemistry (ICC). Suitable for use in Western Blot, Immunoprecipitation and Immunocytochemistry. Dilution: Immunocytochemistry: 1:100 on 4% paraformaldehyde fixed (37 degree C for 30 minutes) cells. Suggested permeabilization method is 0.1% NP40 for 20 minutes. Immunoprecipitation: The preferred tissue/cell lysis buffer is 1% NP40 and/or RIPA. Recommended antibody amount is 3-4ul of ascites. Final reaction volume of 1000ul. Final total protein concentration in reaction mix: 0.1-0.5mg/ml. Suggested incubation time is 16 hours. Sugggested capture is Protein A/G. Western Blot: 1:200. Western blot: 1:200. The antibody reacts with the ~37 kDa torsinA protein. The suggested blocking buffer is TBS with 10% milk and 0.05% Tween-20. The suggested dilution buffer is TBS with 0.05% Tween-20. Recognizes Torsin An at ~3 7kD. Researchers should empirically determine the suitability of the TOR1A tor1a for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Torsin A, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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