The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 2.5 µg/ml. Predicted molecular weight: 62 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
Use a concentration of 4 - 8 µg/ml.
機能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.
組織特異性Highest expression is found in liver followed by skeletal muscle, kidney, heart, brain, placenta, lung and pancreas.
パスウェイAmino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 2/2.
関連疾患Defects 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.
配列類似性Belongs to the aldehyde dehydrogenase family.