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Antibody Product Overview
| ELK.No | ES18407 |
| Product name | AL4A1 rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB |
| Other name | Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial (P5C dehydrogenase) (EC 1.5.1.12) (Aldehyde dehydrogenase family 4 member A1) |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 8659 |
| Human Swiss-Prot | P30038 |
| Source | Rabbit |
| Isotype | IgG |
| Target | AL4A1 |
| Fields | >>Alanine, aspartate and glutamate metabolism;>>Arginine and proline metabolism;>>Metabolic pathways |
| Gene name | ALDH4A1 ALDH4 P5CDH |
| Protein name | AL4A1 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 212647 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q8CHT0 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | P0C2X9 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human AL4A1 AA range: 39-89 |
| Specificity | This antibody detects endogenous levels of AL4A1 at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.103% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 60kD |
| Background | 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. [provided by RefSeq, Jun 2009], |
| Function | catalytic activity:(S)-1-pyrroline-5-carboxylate + NAD(P)(+) + 2 H(2)O = L-glutamate + NAD(P)H.,disease:Defects in ALDH4A1 are the cause of hyperprolinemia type II (HPII) [MIM:239510]. HPII 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.,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.,pathway:Amino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 2/2.,similarity:Belongs to the aldehyde dehydrogena |
| Subcellular location | Mitochondrion matrix. |
| Expression | Highest expression is found in liver followed by skeletal muscle, kidney, heart, brain, placenta, lung and pancreas. |
Additional Images
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Western blot analysis of lysates from A431 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES18407-100
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