Anti-GCDH Antibody (CAB9057)
- SKU:
- CAB9057
- Product type:
- Antibody
- Application:
- WB
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-GCDH Antibody |
| 抗体コード: | CAB9057 |
| 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 149-438 of human GCDH (NP_000150.1). |
| 申し込み: | WB |
| 推奨希釈: | WB 1:200 - 1:2000 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | HL-60, LO2, MCF7, BT-474, Mouse liver, Rat liver, Rat kidney |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 149-438 of human GCDH (NP_000150.1). |
| 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: | MHPI YAYG SEEQ RQKY LPQL AKGE LLGC FGLT EPNS GSDP SSME TRAH YNSS NKSY TLNG TKTW ITNS PMAD LFVV WARC EDGC IRGF LLEK GMRG LSAP RIQG KFSL RASA TGMI IMDG VEVP EENV LPGA SSLG GPFG CLNN ARYG IAWG VLGA SEFC LHTA RQYA LDRM QFGV PLAR NQLI QKKL ADML TEIT LGLH ACLQ LGRL KDQD KAAP EMVS LLKR NNCG KALD IARQ ARDM LGGN GISD EYHV IRHA MNLE AVNT YEGT HDIH ALIL GRAI TGIQ AFTA SK |
| 遺伝子ID: | 2639 |
| Uniprot: | Q92947 |
| セルラーロケーション: | Mitochondrion matrix |
| 計算された分子量: | 47kDa/48kDa |
| 観察された分子量: | 48kDa |
| 同義語: | GCDH, ACAD5, GCD |
| バックグラウンド: | The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. |
| UniProt Protein Function: | GCDH: Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive. Defects in GCDH are the cause of glutaric aciduria type 1 (GA1). GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Lipid Metabolism - fatty acid; Amino Acid Metabolism - tryptophan; Mitochondrial; Oxidoreductase; Amino Acid Metabolism - lysine degradation; EC 1.3.8.6 Chromosomal Location of Human Ortholog: 19p13.2 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:acyl-CoA binding; electron carrier activity; FAD binding; glutaryl-CoA dehydrogenase activity Biological Process: fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; lysine catabolic process Disease: Glutaric Acidemia I |
| NCBI Summary: | The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013] |
| UniProt Code: | Q92947 |
| NCBI GenInfo Identifier: | 2492631 |
| NCBI Gene ID: | 2639 |
| NCBI Accession: | Q92947.1 |
| UniProt Secondary Accession: | Q92947,O14719, A8K2Z2, |
| UniProt Related Accession: | Q92947 |
| Molecular Weight: | 47,355 Da |
| NCBI Full Name: | Glutaryl-CoA dehydrogenase, mitochondrial |
| NCBI Synonym Full Names: | glutaryl-CoA dehydrogenase |
| NCBI Official Symbol: | GCDH |
| NCBI Official Synonym Symbols: | GCD; ACAD5 |
| NCBI Protein Information: | glutaryl-CoA dehydrogenase, mitochondrial |
| UniProt Protein Name: | Glutaryl-CoA dehydrogenase, mitochondrial |
| UniProt Gene Name: | GCDH |
| UniProt Entry Name: | GCDH_HUMAN |
 | Western blot analysis of extracts of various cell lines, using GCDH antibody (CAB9057) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s. |
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