OTC rabbit pAb

• Background :

  This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008]

• Description :

  This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008],

• Product Name Alternative :

  Ornithine carbamoyltransferase, mitochondrial (EC 2.1.3.3) (Ornithine transcarbamylase) (OTCase)

• UniProt :

  P00480

• Swiss Prot :

  P00480

• Reactivity :

  Human; Mouse; Rat

• Immunogen :

  Synthesized peptide derived from human OTC AA range: 275-325

• Target :

  OTC

• Clonality :

  Polyclonal

• Source :

  Rabbit

• Applications :

  WB

• Concentration :

  1 mg/ml

• Dilution :

  WB 1:500-2000

• Buffer :

  -20°C/1 year

• Molecular Weight :

  38kD

• Storage Conditions :

  -20°C/1 year

• Observed Molecular Weight :

  38kD

• Fragment :

  IgG

• Subcellular Location :

  Mitochondrion matrix .

• Other Product Names :

  Ornithine carbamoyltransferase, mitochondrial (EC 2.1.3.3) (Ornithine transcarbamylase) (OTCase)

• Gene ID (Human) :

  5009