Torsin A (Torsin family 1 member A, Dystonia 1 protein)

Cat no: T8067-38

Supplier: United States Biological

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Description
Additional Information

orsinA is an AAA(+) protein that has been demonstrated as an endoplasmic reticulum (ER) chaperone protein involved in a sensitive reporter system for quantitation of processing through the secretory pathway. It is predominantly located in the lumen of the ER and nuclear envelope. TorsinA is responsible for early onset torsion dystonia (DYT1 dystonia), a dominantly inherited movement disorder and disease of basal ganglia function. DYT1 dystonia is commonly caused by the deletion of a glutamic acid (DeltaE) in the carboxyl terminal region of TorsinA, where the protein then aggregates in perinuclear inclusions instead of the ER (1-3). TorsinA is degraded primarily through the macroautophagy

Catalogue number:	T8067-38
Reactivities:	Human
Hosts:	Rabbit
Applications:	Flow Cytometry, Western Blot
Size:	100ul
Form:	Supplied as a liquid in 50 mM Tris-Glycine, pH 7.4, 0.15 M sodium chloride, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.
P type:	Mab
Isotype:	IgG
Purity:	Supernatant
Alternative names:	Torsin family 1 member A, Dystonia 1 protein
References:	1. Hewett JW, et al. Proc Natl Acad Sci U S A. 104(17):7271- 6, 2007 2. Shashidharan P, et al. Brain Res. 853(2):197-206, 2000 3. Torres G E, et al. PNAS 101(44):15650-15655, 2004 4. Giles L M, et al. Human Molecular Genetics 17(17):2712- 2722, 2008
Additional info:	Recognizes human TorsinA.

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Torsin A (Torsin family 1 member A, Dystonia 1 protein)

Cat no: T8067-38

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