GARS Antigen

Cat.No.: DE055

Derived From:
E.coli
Description:
Recombinant Human GARS is produced by E.coli expression system and the target gene encoding MET55-Glu739 is expressed with a 6His tag at the N-terminus.
Accession:
P41250
Names(Known as):
Glycyl tRNA Synthetase, CMT2D, CMT2-D, DSMAV, GlyRS, SMAD1, EJ, Charcot-Marie-Tooth Neuropathy 2D, Glycine tRNA Ligase, Diadenosine tetraphosphate synthetase, AP-4-A synthetase
Quality Control:
Mol Mass:
80.2kDa
AP Mol Mass:
80kDa, reducing conditions
Purity:
Greater than 95% as determined by reducing SDS-PAGE.
Formulation:
Supplied as a 0.2 μM filtered solution of 25mM TrisHCl, 100mM glycine, 10% glycerol, pH7.3.
Shipping:
The product is shipped on dry ice pack. Upon receipt, store it immediately at the temperature listed below.
Storage:
Reconstituted protein solution should be stored at ≤ -20°C.
Purification:
Affinity purification chromatography.
Immunoreactivity:
N.A.
Application:
Immunogen, calibrator or standard.
Background:
Glycyl-tRNA synthetase has been shown to be a target of autoantibodies in the human autoimmune diseases, polymyositis or dermatomyositis.The peripheral nerve diseases Charcot-Marie-Tooth disease type 2D (CMT2D) and distal spinal muscular atrophy type V (dSMA-V) have been liked to dominant mutations in GARS.CMT2D usually manifests during the teenage years, and results in muscle weakness predominantly in the hands and feet.Two mouse models of CMT2D have been used to better understand the disease, identifying that the disorder is caused by a toxic gain-of-function of the mutant glycine-tRNA ligase protein.The CMT2D mice display peripheral nerve axon degeneration and defective development and function of the neuromuscular junction.
FOR RESEARCH OR FURTHER MANUFACTURING USE ONLY

请在下面的搜索框输入货号和批次号,我们会尽快以邮件的形式回复您