The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/50 - 1/100. Detects a band of approximately 62 kDa (predicted molecular weight: 62 kDa).
FunctionIrreversible 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.
Tissue specificityHighest expression is found in liver followed by skeletal muscle, kidney, heart, brain, placenta, lung and pancreas.
PathwayAmino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 2/2.
Involvement in diseaseDefects in ALDH4A1 are the cause of hyperprolinemia type 2 (HP-2) [MIM:239510]. HP-2 is characterized by the accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. The disorder may be causally related to neurologic manifestations, including seizures and mental retardation.
Sequence similaritiesBelongs to the aldehyde dehydrogenase family.