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.

Classification:

Primary antibody

Alternative names:

OTC; Ornithine carbamoyltransferase; mitochondrial; EC 2.1.3.3; Ornithine transcarbamylase; OTCase

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Isotype:

IgG

Immunogen:

Synthesized peptide derived from human OTC

Concentration:

1 mg/ml

Molecular weight:

Applications:

WB

Storage:

-20°C for 1 year

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