Description_x000D_ General description_x000D_ ALDH4A1 (aldehyde dehydrogenase 4 family member A1) belongs to the ALDH family of proteins, and exists as a homodimer. It has a molecular weight of 142-175kDa. This protein is composed of 563 amino acids. It is an NAD+-dependent dehydrogenase localized to the mitochondrial matrix._x000D_ Immunogen_x000D_ Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial Precursor recombinant protein epitope signature tag (PrEST)_x000D_ Application_x000D_ 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._x000D_ 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._x000D_ Physical form_x000D_ Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide_x000D_ Disclaimer_x000D_ Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals._x000D_ Legal Information_x000D_ Prestige Antibodies is a registered trademark of Sigma-Aldrich Co. LLC_x000D_ Biochem/physiol Actions_x000D_ ALDH4A1 (aldehyde dehydrogenase 4 family member A1) catalyzes the oxidation of γ-glutamate semialdehyde to l-glutamate, in an NAD+-dependent manner. It also catalyzes the second step of hydroxyproline catabolism in humans. Mutations in this gene are linked to type II hyperprolinemia (HPII), which is an autosomal recessive disorder, and results in increased levels of ALDH4A1 enzyme and proline in cerebrospinal fluid (CSF), urine and plasma. HPII is characterized by neurological aberrations including seizures, and mental retardation._x000D_ Features and Benefits_x000D_ 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._x000D_ Every Prestige Antibody is tested in the following ways:_x000D_ • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues._x000D_ • Protein array of 364 human recombinant protein fragments._x000D_ • Validated for multiple commonly used applications such as IHC (Immunohistochemistry), IF (Immunofluorescence), and WB (Western Blot)_x000D_ Linkage_x000D_ Corresponding Antigen APREST70261.