OTC rabbit pAb
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 Specifications
Background
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
Available Sizes
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