anti- ALDH4A1 antibody
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Description
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.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | aldehyde dehydrogenase 4 family, member A1 |
| Host | Rabbit |
| Reactivity | human,mouse |
| Recommended Dilution | WB: 1:500 - 1:2000; IHC: 1:50 - 1:200 |
| Clonality | polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Observed MW | 61 kDa |
| Purity | ≥95% as determined by SDS-PAGE |
| Purification | Immunogen affinity purified |
| Size 1 | 100µg |
| Form | liquid |
| Tested Applications | ELISA, WB, IHC |
| Storage | PBS with 0.02% sodium azide and 50% glycerol pH 7.3 , -20℃ for 12 months (Avoid repeated freeze / thaw cycles.) |
| UniProt ID | P30038 |
| Gene ID | 8659 |
| Alias | P5C dehydrogenase |
| Background | Antibody anti-ALDH4A1 |
| Status | RUO |
| Note | This product is for research use only. |
Descripción
ALDH4A1, also known as pyrroline-5-carboxylate dehydrogenase (P5CDH), is a mitochondrial enzyme that catalyzes the conversion of pyrroline-5-carboxylate (P5C) into glutamate as part of the proline degradation pathway. This reaction is critical for maintaining amino acid metabolism, nitrogen balance, and cellular energy production. ALDH4A1 is expressed in tissues such as the liver, kidneys, and muscles, where proline serves as a key energy substrate. Mutations in ALDH4A1 lead to hyperprolinemia type II, a rare metabolic disorder characterized by elevated levels of proline and P5C, causing neurological symptoms, seizures, and cognitive impairments. ALDH4A1 also plays a role in protecting cells from oxidative stress, as proline metabolism generates NADH, which supports mitochondrial function. Its regulation is critical for amino acid catabolism, energy balance, and stress responses under nutrient deprivation.
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