Anti-AASS Antibody (CAB12863)
- SKU:
- CAB12863
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
Frequently bought together:
Description
| 抗体名: | Anti-AASS Antibody |
| 抗体コード: | CAB12863 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 180-400 of human AASS (NP_005754.2). |
| 申し込み: | WB |
| 推奨希釈: | WB 1:500 - 1:2000 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | 293T, Mouse kidney, Mouse liver, Mouse heart, Mouse brain, Rat kidney |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 180-400 of human AASS (NP_005754.2). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | MHIG MAHN YRNS SQAV QAVR DAGY EISL GLMP KSIG PLTF VFTG TGNV SKGA QAIF NELP CEYV EPHE LKEV SQTG DLRK VYGT VLSR HHHL VRKT DAVY DPAE YDKH PERY ISRF NTDI APYT TCLI NGIY WEQN TPRL LTRQ DAQS LLAP GKFS PAGV EGCP ALPH KLVA ICDI SADT GGSI EFMT ECTT IEHP FCMY DADQ HIIH DSVE GSGI LMCS I |
| 遺伝子ID: | 10157 |
| Uniprot: | Q9UDR5 |
| セルラーロケーション: | Mitochondrion |
| 計算された分子量: | 102kDa |
| 観察された分子量: | 102kDa |
 | Western blot - AASS Polyclonal Antibody (CAB12863) |
| 同義語: | AASS, LKR/SDH, LKRSDH, LORSDH |
| バックグラウンド: | 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. |
| 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 |