BCKDK Antibody (PACO44096)
- SKU:
- PACO44096
- 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: | BCKDK Antibody (PACO44096) |
抗体コード: | PACO44096 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC |
推奨される希釈: | ELISA:1:2000-1:10000, WB:1:200-1:1000, IHC:1:20-1:200 |
反応性: | Human |
免疫原: | Recombinant Human [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial protein (153-412AA) |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Western blot. All lanes: BCKDK antibody at 5µg/ml + Hela whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 47, 38, 42 kDa. Observed band size: 47 kDa.. |
![]() | Immunohistochemistry of paraffin-embedded human endometrial cancer using PACO44096 at dilution of 1:100. |
![]() | Immunohistochemistry of paraffin-embedded human breast cancer using PACO44096 at dilution of 1:100. |
バックグラウンド: | Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid, dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex. |
シノニム: | [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial (EC 2.7.11.4) (Branched-chain alpha-ketoacid, dehydrogenase kinase) (BCKD-kinase) (BCKDHKIN), BCKDK |
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 |