3V9G, 3V9H, 3V9I, 4OE58659212647ENSG00000159423ENSMUSG00000028737P30038Q8CHT0NM_001161504NM_003748NM_170726NM_001319218NM_175438NP_001154976NP_001306147NP_003739NP_733844NP_780647Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the ALDH4A1 gene.

[5][6] This protein belongs to the aldehyde dehydrogenase family of proteins.

This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase that 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.

Two transcript variants encoding the same protein have been identified for this gene.