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| Cat.No | ACP10827 | Target Name | AASS |
|---|---|---|---|
| Target Synonyms | AASSAlpha-aminoadipic semialdehyde synthase; mitochondrial; LKR/SDH) [Includes: Lysine ketoglutarate reductase; LKR; LOR; EC 1.5.1.8); Saccharopine dehydrogenase; SDH; EC 1.5.1.9)] | Form | Lyophilized powder |
| Expression System | Custom Production. Please inquire and provide the desire expression system. | Protein Length | Partial |
| Purity | >85% (SDS-PAGE) | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | Q9UDR5 |
|---|
Uniprot Id
Q9UDR5
Target Species
Human
Target Name
AASS
Target Full Name
Alpha-aminoadipic semialdehyde synthase, mitochondrial
Target 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.
Target Involvement
Hyperlysinemia, 1 (HYPLYS1); 2,4-dienoyl-CoA reductase deficiency (DECRD)
Target Subcellular Location
Mitochondrion.
Target Protein Families
AlaDH/PNT family; Saccharopine dehydrogenase family
Target Tissue Specificity
Expressed in all 16 tissues examined with highest expression in the liver.
Target Synonyms
AASSAlpha-aminoadipic semialdehyde synthase; mitochondrial; LKR/SDH) [Includes: Lysine ketoglutarate reductase; LKR; LOR; EC 1.5.1.8); Saccharopine dehydrogenase; SDH; EC 1.5.1.9)]
Target 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.
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