Price range: $268.00 through $328.00
Background:
This gene encodes a bifunctional enzyme that catalyzes the first two steps in the mammalian lysine degradation pathway. The N-terminal and the C-terminal portions of this enzyme contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively, resulting in the conversion of lysine to alpha-aminoadipic semialdehyde. Mutations in this gene are associated with familial hyperlysinemia. [provided by RefSeq, Jul 2008],
| Size | 50μL/100μL |
| Target | AASS |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, ELISA |
| MW(Calculated) | |
| MW(Observed) | 102kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; ELISA 1:10000; Not yet tested in other applications. |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | The antiserum was produced against synthesized peptide derived from human AASS. AA range:251-300 |
| Specificity | AASS Polyclonal Antibody detects endogenous levels of AASS protein. |
| Gene Name | AASS |
| Protein Name | Alpha-aminoadipic semialdehyde synthase mitochondrial |
| Other Name | AASS; / Alpha-aminoadipic semialdehyde synthase; / mitochondrial; / LKR/SDH |
| Species | Human |
| Gene ID | 10157 |
| Uniprot | Q9UDR5, |
| Species.1 | Mouse |
| Gene ID.1 | |
| Uniprot.1 | Q99K67 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Mitochondrion . |
| Tissue Expression | Expressed in all 16 tissues examined with highest expression in the liver. |
| Signaling_pathway | |
| Research Areas | >>Lysine degradation; / >>Metabolic pathways |
| Function | Catalytic activity:N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD(+) + H(2)O = L-glutamate + 2-aminoadipate 6-semialdehyde + NADH.,Catalytic activity:N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP(+) + H(2)O = L-lysine + 2-oxoglutarate + NADPH.,Disease:Defects in AASS are the cause of hyperlysinemia [MIM:238700]. Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria.,Function:Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.,induction:Induced by starvation.,pathway:Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 1/6.,pathway:Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 2/6.,similarity:In the C-terminal section; belongs to the saccharopine dehydrogenase family.,similarity:In the N-terminal section; belongs to the AlaDH/PNT family.,subunit:Homodimer.,tissue specificity:Expressed in all 16 tissues examined with highest expression in the liver., |
