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OTC rabbit pAb
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ES14359
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50 µL $148.00
100 µL $248.00
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Overview

Product name: OTC rabbit pAb
Reactivity: Human; Mouse;Rat
Alternative Names: Ornithine carbamoyltransferase, mitochondrial (EC 2.1.3.3) (Ornithine transcarbamylase) (OTCase)
Source: Rabbit
Dilutions: WB 1:500-2000
Immunogen: Synthesized peptide derived from human OTC AA range: 275-325
Storage: -20°C/1 year
Clonality: Polyclonal
Isotype: IgG
Concentration: 1 mg/ml
Observed Band: 38kD
GeneID: 5009
Human Swiss-Prot No: P00480
Cellular localization: Mitochondrion matrix .
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],