Información del producto
Información adicional
Storage Conditions
-20°C/1 year
Applications
WB
Concentration
1 mg/ml
Recomended Dilution
WB 1:500-2000
Type Clonality
Polyclonal
Immunogen
Synthesized peptide derived from human OTC AA range: 275-325
Subcellular Location
Mitochondrion matrix .
Iso type
IgG
Tissue Source
Rabbit
Reactivity
Human
Mouse
Rat
Mouse
Rat
Human Gen
5009
Observed Band
38kD
Other Name
Ornithine carbamoyltransferase, mitochondrial (EC 2.1.3.3) (Ornithine transcarbamylase) (OTCase)
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