产品说明

一般描述

The gene LIAS (lipoyl synthase - mitochondrial) is mapped to human chromosome 4p14. The protein is mainly present in the mitochondria.

免疫原

Lipoyl synthase, mitochondrial precursor recombinant protein epitope signature tag (PrEST)

应用

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org)and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.

生化/生理作用

Lipoyl synthase - mitochondrial (LIAS) is involved in synthesis of lipoic acid, a mitochondrial antioxidant. Absence of LIAS leads to imbalance in the antioxidant defense system, thereby causing inflammation, insulin resistance and mitochondrial abnormalities. Mutations in LIAS are associated with nonketotic hyperglycinemia, characterized by deficiency in lipoylation of mitochondrial proteins. In addition, mutation is LIAS is linked with neonatal-onset epilepsy, muscular hypotonia, lactic acidosis and high glycine levels in plasma and urine.

特点和优势

Prestige Antibodies^® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies^® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:

• IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
• Protein array of 364 human recombinant protein fragments.

联系

Corresponding Antigen APREST74695.

外形

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

法律信息

Prestige Antibodies is a registered trademark of sigma-aldrich Co. LLC

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