AASS Antibody (PACO59465)
- SKU:
- PACO59465
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | AASS Antibody (PACO59465) |
抗体コード: | PACO59465 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC |
推奨される希釈: | ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:200-1:500 |
反応性: | Human |
免疫原: | Recombinant Human Alpha-aminoadipic semialdehyde synthase, mitochondrial protein (224-364AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Western Blot. Positive WB detected in: Hela whole cell lysate, 293T whole cell lysate, HepG2 whole cell lysate. All lanes: AASS antibody at 5.8µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 103 kDa. Observed band size: 103 kDa. |
![]() | IHC image of PACO59465 diluted at 1:400 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
![]() | IHC image of PACO59465 diluted at 1:400 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
バックグラウンド: | 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. |
シノニム: | Alpha-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)], AASS |
UniProt Protein Function: | AASS: 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. Defects in AASS are the cause of hyperlysinemia (HYPLYS). Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria. |
UniProt Protein Details: | Protein type:Amino Acid Metabolism - lysine degradation; EC 1.5.1.8; Oxidoreductase; Mitochondrial; Amino Acid Metabolism - lysine biosynthesis; EC 1.5.1.9 Chromosomal Location of Human Ortholog: 7q31.3 Cellular Component: intracellular membrane-bound organelle; mitochondrial matrix; mitochondrion Molecular Function:saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity; saccharopine dehydrogenase (NADP+, L-lysine-forming) activity Biological Process: lysine catabolic process; protein tetramerization Disease: Hyperlysinemia, Type I; Saccharopinuria |
NCBI Summary: | 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] |
UniProt Code: | Q9UDR5 |
NCBI GenInfo Identifier: | 46396032 |
NCBI Gene ID: | 10157 |
NCBI Accession: | Q9UDR5.1 |
UniProt Secondary Accession: | Q9UDR5,O95462, |
UniProt Related Accession: | Q9UDR5 |
Molecular Weight: | 102,132 Da |
NCBI Full Name: | Alpha-aminoadipic semialdehyde synthase, mitochondrial |
NCBI Synonym Full Names: | aminoadipate-semialdehyde synthase |
NCBI Official Symbol: | AASS |
NCBI Official Synonym Symbols: | LKRSDH; LORSDH; LKR/SDH |
NCBI Protein Information: | alpha-aminoadipic semialdehyde synthase, mitochondrial |
UniProt Protein Name: | Alpha-aminoadipic semialdehyde synthase, mitochondrial |
UniProt Synonym Protein Names: | LKR/SDHIncluding the following 2 domains:Lysine ketoglutarate reductase (EC:1.5.1.8); LKR; LORSaccharopine dehydrogenase (EC:1.5.1.9); SDH |
UniProt Gene Name: | AASS |
UniProt Entry Name: | AASS_HUMAN |