Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations
- Western blot [1]
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- Product number
- BS8180 - Provider product page
- Provider
- Bioworld Technology, Inc
- Product name
- OGDH polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- Recombinant full length Human OGDH.
- Description
- The Ï«-ketoglutarate dehydrogenase (Ï«-KGD) complex is a multienzyme complex which localizes to the mitochondrial matrix and consists of three protein subunits: Ï«-ketoglutarate dehydrogenase, also designated Ï«-KGD, E1k or oxoglutarate dehydrogenase (OGDH); dihydrolipoyl succinyltransferase (E2k or DLST); and dihydrolipoyl dehydrogenase (E3). The Ï«-KGD subunit of the Ï«-KGD complex catalyzes the conversion of Ï«-ketoglutarate to succinyl-CoA and CO2, an essential reaction of the tricarboxylic acid cycle. A definciency in Ï«-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.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
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Supportive validation
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- Bioworld Technology, Inc (provider)
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- Experimental details
- WesternBlot (WB) analysis of OGDH polyclonal antibody