Human Glycyl-tRNA synthetase (GARS) ELISA kit; CMT2D; DSMAV; GlyRS; HMN5; SMAD1; Charcot-Marie-Tooth neuropathy 2D; Charcot-Marie-Tooth neuropathy; neuronal type; D; glycine tRNA ligase; glycyl-tRNA synthetase

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

This assay has high sensitivity and excellent specificity for detection of Human GARS. No significant cross-reactivity or interference between Human GARS and analogues was observed.

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of GARS elisa kit 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.

MBS909109 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the glycyl-tRNA synthetase (GARS) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing GARS. The ELISA analytical biochemical technique of the MBS909109 kit is based on GARS antibody-GARS antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect GARS antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, GARS. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for GARS has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any GARS present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for GARS is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of GARS bound in the initial step. The color development is stopped and the intensity of the color is measured

83,166 Da

glycyl-tRNA synthetase

glycine--tRNA ligase; AP-4-A synthetase; glycine tRNA ligase; Charcot-Marie-Tooth neuropathy 2D; diadenosine tetraphosphate synthetase; Charcot-Marie-Tooth neuropathy, neuronal type, D

Glycine--tRNA ligase

AP-4-A synthetase; GlyRS??[Similar Products]

SYG_HUMAN

This gene encodes glycyl-tRNA synthetase, one 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. [provided by RefSeq, Jul 2008]

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; Translation; Mitochondrial

Chromosomal Location of Human Ortholog: 7p15

Cellular Component: nucleoplasm; mitochondrial matrix; cytoplasm; cytosol; secretory granule

Molecular Function: protein dimerization activity; glycine-tRNA ligase activity; ATP binding

Biological Process: tRNA aminoacylation for protein translation; glycyl-tRNA aminoacylation; gene expression; diadenosine tetraphosphate biosynthetic process

Disease: Neuronopathy, Distal Hereditary Motor, Type Va; Charcot-marie-tooth Disease, Axonal, Type 2d

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