|Other Names||Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial, P5C dehydrogenase, Aldehyde dehydrogenase family 4 member A1, L-glutamate gamma-semialdehyde dehydrogenase, ALDH4A1, ALDH4, P5CDH|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP7875c was selected from the Center region of human ALDH4A1. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Irreversible conversion of delta-1-pyrroline-5- carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes.|
|Cellular Location||Mitochondrion matrix.|
|Tissue Location||Highest expression is found in liver followed by skeletal muscle, kidney, heart, brain, placenta, lung and pancreas|
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Provided below are standard protocols that you may find useful for product applications.
ALDH4A1 belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline.
Yoon,K.A., J. Hum. Genet. 49 (3), 134-140 (2004)Geraghty,M.T., Hum. Mol. Genet. 7 (9), 1411-1415 (1998)
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