DYT1, Monoclonal Antibody

Torsin-1A; Dystonia 1 protein; Torsin ATPase-1A; 364-; Torsin family 1 member A; TOR1A; DQ2; DYT1; TA; TORA

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

IgG1

583CT1.1.3

Mouse

Purified His-tagged DYT1 protein(Fragment) was used to produced this monoclonal antibody.

Mouse monoclonal antibody supplied in crude ascites with 0.09% (W/V) sodium azide.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-TOR1A antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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].

Neuroscience

Western Blot (WB), ELISA (EIA)

WB~~1:500~8000

DYT1 Antibody western blot analysis in ZR-75-1 cell line lysates (35ug/lane).This demonstrates the DYT1 antibody detected the DYT1 protein (arrow).

37809

torsin family 1, member A (torsin A)

torsin-1A

Torsin-1A

DQ2; DYT1; TA; TORA  [Similar Products]

TOR1A_HUMAN

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]

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; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 9q34

Cellular Component: synaptic vesicle; nuclear membrane; transport vesicle; cytoplasmic vesicle membrane; cytoskeleton; growth cone; membrane; intracellular membrane-bound organelle; endoplasmic reticulum lumen; nuclear envelope; cell junction; secretory granule

Molecular Function: protein binding; kinesin binding; unfolded protein binding; ATPase activity; cytoskeletal 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; protein homooligomerization; nuclear membrane organization and biogenesis; neurite development

Disease: Dystonia 1, Torsion, Autosomal Dominant

Sharma, N., et al. Mov. Disord. 25(13):2183-2187(2010)
Kaiser, F.J., et al. Ann. Neurol. 68(4):554-559(2010)
Gavarini, S., et al. Ann. Neurol. 68(4):549-553(2010)
Granata, A., et al. Eur. J. Neurol. 17 SUPPL 1, 81-87 (2010) :
Warner, T.T., et al. Biochem. Soc. Trans. 38(2):452-456(2010)

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