antibody

Mouse Anti Human Glycyl-TRNA Synthetase

MBS140491

0.005 mg

140 USD

monoclonal

mouse

nonconjugated

[PAT4E10AT]

Antibody

Mouse Anti Human Glycyl-TRNA Synthetase

[Glycyl-TRNA Synthetase]

[GARS Antibody; Glycyl-TRNA Synthetase, Mouse Anti Human; Glycine--tRNA ligase; Diadenosine tetraphosphate synthetase; AP-4-A synthetase; Glycyl-tRNA synthetase; GlyRS; GARS; CMT2D; DSMAV; HMN5; SMAD1]

[glycyl tRNA synthetase; Glycine--tRNA ligase; glycine--tRNA ligase; glycyl-tRNA synthetase; Diadenosine tetraphosphate synthetase; AP-4-A synthetase; Glycyl-tRNA synthetase; GlyRS]

[GARS]

[GARS; GARS; HMN5; CMT2D; DSMAV; GlyRS; SMAD1; AP-4-A synthetase; GlyRS]

SYG_HUMAN

m

Mouse IgG1 heavy chain and k light chain.

[PAT4E10AT]

Mouse

Human

739

GARS antibody was purified from mouse ascitic fluids by protein -A affinity chromatography.

1mg/ml containing PBS, pH-7.4, 10% Glycerol and 0.02% Sodium Azide. Sterile filtered colorless solution.

Storage: For periods up to 1 month store at 4°C, for longer periods of time, store at -20°C. Prevent freeze thaw cycles. Stability: 12 months at -20°C. 1 month at 4°C.

GARS antibody has been tested by ELISA, Western blot analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results.

Recommended starting dilution is 1:500

Type: Mouse Anti Human Monoclonal. Immunogen: Anti-human GARS mAb, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with recombinant human GARS amino acids 43-289 purified from E Coli.

MONOCLONAL ANTIBODIES; ANTI-HUMAN ENZYMES

GARS is an (alpha)2 dimer which is a member of the class II family of tRNA synthetases. GARS is a glycyl-tRNA synthetase, one of the aminoacyl-tRNA synthetases which charge tRNAs with their cognate amino acids. GARS catalyzes the attachment of glycine to tRNA(Gly). In addition, GARS is able to produce diadenosine tetraphosphate (Ap4A), which is a universal pleiotropic signaling molecule required for cell regulation pathways, by direct condensation of two ATPs. GARS has been demonstrated to be a target of autoantibodies in the human autoimmune diseases, polymyositis or dermatomyositis.

1311463

BAA06338.1

Aminoacyl-tRNA Biosynthesis Pathway (83030); Aminoacyl-tRNA Biosynthesis Pathway (424); Aminoacyl-tRNA Biosynthesis, Eukaryotes Pathway (413439); Aminoacyl-tRNA Biosynthesis, Eukaryotes Pathway (468360); Cytosolic TRNA Aminoacylation Pathway (1269710); Gene Expression Pathway (1269649); Mitochondrial TRNA Aminoacylation Pathway (1269711); TRNA Aminoacylation Pathway (1269709)

GARS: Catalyzes the attachment of glycine to tRNA(Gly). Is also able produce diadenosine tetraphosphate (Ap4A), a universal pleiotropic signaling molecule needed for cell regulation pathways, by direct condensation of 2 ATPs. Defects in GARS are the cause of Charcot-Marie-Tooth disease type 2D (CMT2D). CMT2D is a form of Charcot- Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2D is characterized by a more severe phenotype in the upper extremities (severe weakness and atrophy, absence of tendon reflexes) than in the lower limbs. CMT2D inheritance is autosomal dominant. Defects in GARS are a cause of distal hereditary motor neuronopathy type 5A (HMN5A); also known as distal hereditary motor neuropathy type V (DSMAV). A disorder characterized by distal muscular atrophy mainly affecting the upper extremities, in contrast to other distal motor neuronopathies. These constitute a heterogeneous group of neuromuscular diseases caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs. Belongs to the class-II aminoacyl-tRNA synthetase family. Protein type: EC 6.1.1.14; Ligase; Mitochondrial; Translation. Chromosomal Location of Human Ortholog: 7p15. Cellular Component: axon; cytoplasm; cytosol; mitochondrial matrix; nucleoplasm. Molecular Function: bis(5'-nucleosyl)-tetraphosphatase (asymmetrical) activity; glycine-tRNA ligase activity; protein dimerization activity. Biological Process: diadenosine tetraphosphate biosynthetic process; glycyl-tRNA aminoacylation; tRNA aminoacylation for protein translation. Disease: Charcot-marie-tooth Disease, Axonal, Type 2d; Neuronopathy, Distal Hereditary Motor, Type Va

0.005 mg

140 USD

0.02 mg

205

0.1 mg

505