Anti-AUH Antibody (CAB7424)
- SKU:
- CAB7424
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Frequently bought together:
Description
抗体名: | Anti-AUH Antibody |
抗体コード: | CAB7424 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 68-339 of human AUH (NP_001689.1). |
申し込み: | WB IHC |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | A-549, SW480, HepG2, Mouse brain, Mouse heart, Mouse kidney, Rat brain, Rat heart, Rat kidney |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 68-339 of human AUH (NP_001689.1). |
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: | SSEM KTED ELRV RHLE EENR GIVV LGIN RAYG KNSL SKNL IKML SKAV DALK SDKK VRTI IIRS EVPG IFCA GADL KERA KMSS SEVG PFVS KIRA VIND IANL PVPT IAAI DGLA LGGG LELA LACD IRVA ASSA KMGL VETK LAII PGGG GTQR LPRA IGMS LAKE LIFS ARVL DGKE AKAV GLIS HVLE QNQE GDAA YRKA LDLA REFL PQGP VAMR VAKL AINQ GMEV DLVT GLAI EEAC YAQT IPTK DRLE GLLA FKEK RPPR YKGE |
遺伝子ID: | 549 |
Uniprot: | Q13825 |
セルラーロケーション: | Mitochondrion |
計算された分子量: | 32kDa/35kDa |
観察された分子量: | 30kDa |
同義語: | AUH |
バックグラウンド: | This gene encodes bifunctional mitochondrial protein that has both RNA-binding and hydratase activities. The encoded protein is a methylglutaconyl-CoA hydratase that catalyzes the hydration of 3-methylglutaconyl-CoA to 3-hydroxy-3-methyl-glutaryl-CoA, a critical step in the leucine degradation pathway. This protein also binds AU-rich elements (AREs) found in the 3' UTRs of rapidly decaying mRNAs including c-fos, c-myc and granulocyte/ macrophage colony stimulating factor. ARE elements are involved in directing RNA to rapid degradation and deadenylation. This protein is localizes to the mitochondrial matrix and the inner mitochondrial membrane and may be involved in mitochondrial protein synthesis. Mutations in this gene are the cause of 3-methylglutaconic aciduria, type I. Alternative splicing results in multiple transcript variants. |
UniProt Protein Function: | AUH: Catalyzes the conversion of 3-methylglutaconyl-CoA to 3- hydroxy-3-methylglutaryl-CoA. Has very low enoyl-CoA hydratase activity. Was originally identified as RNA-binding protein that binds in vitro to clustered 5'-AUUUA-3' motifs. Defects in AUH are the cause of 3-methylglutaconic aciduria type 1 (MGA1). MGA1 is an inborn error of leucine metabolism. It leads to an autosomal recessive syndrome with variable clinical phenotype, ranging from delayed speech development to severe psychomotor retardation, coma, failure to thrive, metabolic acidosis and dystonia. MGA1 can be distinguished from other forms of MGA by the pattern of metabolite excretion: 3- methylglutaconic acid levels are higher than those detected in other forms, whereas methylglutaric acid levels are usually only slightly elevated, and there is a high level of 3- hydroxyisovaleric acid excretion (not present in other MGA forms). Belongs to the enoyl-CoA hydratase/isomerase family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Mitochondrial; Lyase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 4.2.1.18; RNA-binding Chromosomal Location of Human Ortholog: 9q22.31 Cellular Component: mitochondrion; mitochondrial matrix Molecular Function:methylglutaconyl-CoA hydratase activity; mRNA 3'-UTR binding; enoyl-CoA hydratase activity Biological Process: leucine catabolic process; branched chain family amino acid catabolic process Disease: 3-methylglutaconic Aciduria, Type I |
NCBI Summary: | This gene encodes bifunctional mitochondrial protein that has both RNA-binding and hydratase activities. The encoded protein is a methylglutaconyl-CoA hydratase that catalyzes the hydration of 3-methylglutaconyl-CoA to 3-hydroxy-3-methyl-glutaryl-CoA, a critical step in the leucine degradation pathway. This protein also binds AU-rich elements (AREs) found in the 3' UTRs of rapidly decaying mRNAs including c-fos, c-myc and granulocyte/ macrophage colony stimulating factor. ARE elements are involved in directing RNA to rapid degradation and deadenylation. This protein is localizes to the mitochondrial matrix and the inner mitochondrial membrane and may be involved in mitochondrial protein synthesis. Mutations in this gene are the cause of 3-methylglutaconic aciduria, type I. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2015] |
UniProt Code: | Q13825 |
NCBI GenInfo Identifier: | 37076898 |
NCBI Gene ID: | 549 |
NCBI Accession: | Q13825.1 |
UniProt Related Accession: | Q13825 |
Molecular Weight: | 37kDa |
NCBI Full Name: | Methylglutaconyl-CoA hydratase, mitochondrial |
NCBI Synonym Full Names: | AU RNA binding methylglutaconyl-CoA hydratase |
NCBI Official Symbol: | AUH |
NCBI Protein Information: | methylglutaconyl-CoA hydratase, mitochondrial |
UniProt Protein Name: | Methylglutaconyl-CoA hydratase, mitochondrial |
UniProt Synonym Protein Names: | AU-specific RNA-binding enoyl-CoA hydratase; AU-binding protein/enoyl-CoA hydratase |
Protein Family: | Methylglutaconyl-CoA hydratase |
UniProt Gene Name: | AUH |
UniProt Entry Name: | AUHM_HUMAN |
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