OGDH Antilichaam - N-terminalgebied: FITC (ARP58648_P050-FITC)

Oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)

E1k, KGD1, OGDC, AKGDH, OGDH2

4967

Q96DD3

NP_001003941

Human, Mouse, Rat, Cow, Guinea Pig, Horse, Rabbit

The immunogen is a synthetic peptide directed towards the N terminal region of human OGDH

The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO2. It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) .This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzes the overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO (2) during the Krebs cycle. The protein is located in the mitocondrial matrix and uses thiamine pyrophosphate as a cofactor. A congential deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia.

MDM2; UBC; SUMO1; NEDD8; CPSF7; MRPL38; MRPL41; TSR1; MRPS2; MRPL15; MRPL13; SCFD1; PDHX; SSBP1; SUMO2; PDHB; PDHA1; DLST; DLAT; DHODH; ISG15; USP13; PLSCR1; DLD

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 100%; Guinea Pig: 93%; Horse: 100%; Human: 100%; Mouse: 93%; Rabbit: 93%; Rat: 93%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

47kDa

Van (2007) Am. J. Med. Genet. A 143 (7), 763-767

Wet Ice

427

OGDH

OGDH

Rabbit

2-oxoglutarate dehydrogenase, mitochondrial

NM_001003941

MFHLRTCAAKLRPLTASQTVKTFSQNRPAAARTFQQIRCYSAPVAAEPFL