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363221

GARS (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)

Size

£499.00

SKU:
363221
Purity:
Purified by Protein A and peptide affinity chromatography
Storage Conditions:
-20°C
Supplier:
United States Biological
Host:
Rabbit
Immunogen:
Recombinant protein corresponding to Val567-Glu735 of human GARS, expressed in E. coli
Format:
Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol
Extra Details:
Glycyl-tRNA synthetase isone of the aminoacyl-tRNA synthetases that charge tRNAs with their cognate amino acids. The encoded enzyme is an (alpha)2 dimer which belongs to the class II family of tRNA synthetases. It has been shown to be a target of autoantibodies in the human autoimmune diseases, polymyositis or dermatomyositis.Defects in GARS are the cause of distal spinal muscular atrophy type V (DSMA-V). DSMA-V is an autosomal dominant distal hereditary motor neuropathy (dHMN) with a phenotype similar to CMTD2. The main characteristic that distinguishes these disorders is the less severe distal sensory involvement in DSMA-V patients.Defects in GARS are the cause of Charcot-Marie-Tooth disease type 2D (CMT2D). Applications: Suitable for use in ELISA, Western Blot, Immunohistochemistry and Immunocytochemistry. Other applications not tested. Recommended Dilutions: Western Blot: 0.5-2ug/ml Immunohistochemistry (Paraffin): 5-30ug/ml Immunocytochemistry: 5-30ug/ml Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Shipping Conditions:
Blue Ice