Glycine cleavage system H protein, ELISA Kit

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

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

Store at 2-8 degree C.

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 GCSH 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.

MBS282908 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Glycine cleavage system H protein (GCSH) 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 GCSH. The ELISA analytical biochemical technique of the MBS282908 kit is based on GCSH antibody-GCSH antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect GCSH antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, GCSH. 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 a two-site sandwich ELISA to quantitate GCSH in samples. An antibody specific for GCSH has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyGCSH present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for GCSH is added to the wells. After washing, Streptavidin 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 GCSH bound in the initial step. The color development is stopped and the intensity of the color is measured.

18,885 Da

glycine cleavage system protein H

glycine cleavage system H protein, mitochondrial

Glycine cleavage system H protein, mitochondrial

Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.[provided by RefSeq, Jan 2010]

GCSH: The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein. Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH); also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Belongs to the GcvH family.

Chromosomal Location of Human Ortholog: 16q23.2

Cellular Component: glycine cleavage complex; mitochondrial matrix; mitochondrion

Molecular Function: aminomethyltransferase activity; protein binding

Biological Process: glycine catabolic process; glycine decarboxylation via glycine cleavage system; methylation; protein lipoylation

Disease: Glycine Encephalopathy

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