Anti-BCKDK Antibody (CAB8184)
- SKU:
- CAB8184
- Product type:
- Antibody
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | BCKDK Rabbit Polyclonal Antibody |
| 抗体コード: | CAB8184 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IHC IF |
| 反応性: | Human, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 153-412 of human BCKDK (NP_005872.2). |
| 申し込み: | WB IHC IF |
| 推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100 |
| 反応性: | Human, Rat |
| ポジティブサンプル: | 22Rv1 |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 153-412 of human BCKDK (NP_005872.2). |
| 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: | QLVR QLLD DHKD VVTL LAEG LRES RKHI EDEK LVRY FLDK TLTS RLGI RMLA THHL ALHE DKPD FVGI ICTR LSPK KIIE KWVD FARR LCEH KYGN APRV RING HVAA RFPF IPMP LDYI LPEL LKNA MRAT MESH LDTP YNVP DVVI TIAN NDVD LIIR ISDR GGGI AHKD LDRV MDYH FTTA EAST QDPR ISPL FGHL DMHS GAQS GPMH GFGF GLPT SRAY AEYL GGSL QLQS LQGI GTDV YLRL RHID GREE SFRI |
| 遺伝子ID: | 10295 |
| Uniprot: | O14874 |
| セルラーロケーション: | Mitochondrion, Mitochondrion matrix |
| 計算された分子量: | 37kDa/41kDa/46kDa |
| 観察された分子量: | 46kDa |
| 同義語: | BCKDK, BCKDKD, BDK |
| バックグラウンド: | The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene. |
| UniProt Protein Function: | BCKDK: an atypical protein kinase associated with the mitochondrial matrix. Contains a HATPase_c domain, found in several ATP-binding proteins including protein histidine kinases (PHKs), PHDKs, DNA gyrase B, topoisomerases, heat shock proteins, and DNA mismatch repair proteins. Unlike PHKs, BCK dimerizes through direct interaction of two opposing nucleotide-binding domains. Phosphorylates and inactivates the E1-alpha subunit of the branched-chain ketoacid dehydrogenase (BCKDK), the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Inactivating mutations have been discovered in BCKDK in families with autism, epilepsy, and intellectual disability, a syndrome that may respond to dietary supplementation. |
| UniProt Protein Details: | Protein type:Mitochondrial; Kinase, protein; Protein kinase, atypical; EC 2.7.11.4; ATYPICAL group; PDHK family Chromosomal Location of Human Ortholog: 16p11.2 Cellular Component: mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrion Molecular Function:[3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity; ATP binding; kinase activity; protein binding; protein serine/threonine kinase activity Biological Process: amino acid catabolic process; branched chain family amino acid catabolic process; phosphorylation; protein amino acid phosphorylation Disease: Branched-chain Ketoacid Dehydrogenase Kinase Deficiency |
| NCBI Summary: | The branched-chain alpha-ketoacid dehydrogenase complex (BCKD) is an important regulator of the valine, leucine, and isoleucine catabolic pathways. The protein encoded by this gene is found in the mitochondrion, where it phosphorylates and inactivates BCKD. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2012] |
| UniProt Code: | O14874 |
| NCBI GenInfo Identifier: | 21431746 |
| NCBI Gene ID: | 10295 |
| NCBI Accession: | O14874.2 |
| UniProt Secondary Accession: | O14874,Q6FGL4, Q96G95, Q96IN5, A8MY43, |
| UniProt Related Accession: | O14874 |
| Molecular Weight: | 41,090 Da |
| NCBI Full Name: | [3-methyl-2-oxobutanoate dehydrogenase |
| NCBI Synonym Full Names: | branched chain ketoacid dehydrogenase kinase |
| NCBI Official Symbol: | BCKDK |
| NCBI Official Synonym Symbols: | BDK; BCKDKD |
| NCBI Protein Information: | [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial |
| UniProt Protein Name: | [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial |
| UniProt Synonym Protein Names: | Branched-chain alpha-ketoacid dehydrogenase kinase; BCKD-kinase; BCKDHKIN |
| UniProt Gene Name: | BCKDK |
| UniProt Entry Name: | BCKD_HUMAN |
 | Western blot analysis of extracts of 22Rv1 cells, using BCKDK antibody (CAB8184) 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: 90s. |
 | Immunohistochemistry of paraffin-embedded rat lung using BCKDK antibody (CAB8184) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded rat kidney using BCKDK antibody (CAB8184) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded human liver damage using BCKDK antibody (CAB8184) at dilution of 1:100 (40x lens). |
 | Immunofluorescence analysis of U2OS cells using BCKDK antibody (CAB8184) at dilution of 1:100. Blue: DAPI for nuclear staining. |
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