DBT Antibody (PACO60681)
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주문- SKU:
- PACO60681
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | DBT Antibody (PACO60681) |
Antibody SKU: | PACO60681 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:5000 |
Species Reactivity: | Human, Mouse |
Immunogen: | Recombinant Human Lipoamide acyltransferase component of branched-chain alpha-keto acid, dehydrogenase complex, mitochondrial protein (140-259AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western Blot. Positive WB detected in: 293 whole cell lysate, Mouse kidney tissue, Mouse liver tissue. All lanes: DBT antibody at 4.3µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 54 kDa. Observed band size: 54 kDa. |
Background: | The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acid, to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid, decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid, decarboxylase component. |
Synonyms: | Lipoamide acyltransferase component of branched-chain alpha-keto acid, dehydrogenase complex, mitochondrial (EC 2.3.1.168) (52 kDa mitochondrial autoantigen of primary biliary cirrhosis) (Branched chain 2-oxo-acid, dehydrogenase complex component E2) (BCOADC-E2) (Branched-chain alpha-keto acid, dehydrogenase complex component E2) (BCKAD-E2) (BCKADE2) (Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid, dehydrogenase complex) (Dihydrolipoamide branched chain transacylase) (Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase), DBT, BCATE2 |
UniProt Protein Function: | DBT: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in DBT are the cause of maple syrup urine disease type 2 (MSUD2). MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine. Belongs to the 2-oxoacid dehydrogenase family. |
UniProt Protein Details: | Protein type:Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.1.168; Mitochondrial; Transferase Chromosomal Location of Human Ortholog: 1p31 Cellular Component: mitochondrion; mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix Molecular Function:dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity Biological Process: branched chain family amino acid catabolic process Disease: Maple Syrup Urine Disease |
NCBI Summary: | The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq, Jul 2008] |
UniProt Code: | P11182 |
NCBI GenInfo Identifier: | 400668 |
NCBI Gene ID: | 1629 |
NCBI Accession: | P11182.3 |
UniProt Related Accession: | P11182 |
Molecular Weight: | Observed Molecular Weight: (Observed)53kD.Predicted Molecular Weight: (Calculated)53kDa. |
NCBI Full Name: | Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial |
NCBI Synonym Full Names: | dihydrolipoamide branched chain transacylase E2 |
NCBI Official Symbol: | DBT |
NCBI Official Synonym Symbols: | E2; E2B; BCATE2; BCKADE2; BCKAD-E2; BCOADC-E2 |
NCBI Protein Information: | lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial |
UniProt Protein Name: | Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial |
UniProt Synonym Protein Names: | 52 kDa mitochondrial autoantigen of primary biliary cirrhosis; Branched chain 2-oxo-acid dehydrogenase complex component E2 |
UniProt Gene Name: | DBT |
UniProt Entry Name: | ODB2_HUMAN |
Antibodies | ELISA Kits |
DBT Antibody (PACO08754) | Human DBT ELISA Kit |
Human Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial (DBT) ELISA Kit |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
Recommended Products |
Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
Anti-HRP-conjugated Beta Actin Antibody (CABC028) |