| Product Name | α-KGD Antibody (H-61) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 1-61 mapping at the N-terminus of α-KGD of human origin |
| Modification Notes | The å-ketoglutarate dehydrogenase (å-KGD) complex is a multienzyme complex which localizes to the mitochondrial matrix and consists of three protein subunits: å-ketoglutarate dehydrogenase, also designated å-KGD, E1k or oxoglutarate dehydrogenase (OGDH); dihydrolipoyl succinyltransferase (E2k or DLST); and dihydrolipoyl dehydrogenase (E3). The å-KGD subunit of the å-KGD complex catalyzes the conversion of å-ketoglutarate to succinyl-CoA and CO2, an essential reaction of the tricarboxylic acid cycle. A definciency in å-KGD results in hypotonia, metabolic acidosis, hyperlactatemia immediately after birth, and neurologic deterioration resulting in death at about 30 months of age. Low molar ratios of ketone bodies in plasma of neonates with congenital lactic acidosis are proposed indicators of tricarboxylic acid cycle dysfunction. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of α-KGD of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, bovine, porcine and avian: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 1-61 mapping at the N-terminus of &alpha -KGD of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 1-61 mapping at the N-terminus of α-KGD of human origin |
| Entrez Gene | 4967 |
|---|
| Application Notes | recommended for detection of α-KGD of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, bovine, porcine and avian: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
