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This protein 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. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. Protein 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. [The UniProt Consortium]
Keywords:
Anti-ALDH4, Anti-ALDH4A1, EC=1.2.1.88, Anti-P5C dehydrogenase, Anti-Aldehyde dehydrogenase family 4 member A1, Anti-L-glutamate gamma-semialdehyde dehydrogenase, Anti-Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial, ALDH4A1 Polyclonal Antibod
This website uses cookies, which are necessary for the technical operation of the website and are always set. Other cookies, which increase the usability of this website, serve for direct advertising or simplify interaction with other websites and social networks, will only be used with your consent.
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