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| Cat.No | ACP22289 | Target Name | OTC |
|---|---|---|---|
| Target Synonyms | EC 2.1.3.3; MGC129967; MGC129968; MGC138856; OCTD; Ornithine Carbamoyltransferase; Ornithine carbamoyltransferase mitochondrial; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase | Form | Lyophilized powder |
| Expression System | Custom Production. Please inquire and provide the desire expression system. | Expression Range | 33-354 |
| Protein Length | Full Length of Mature Protein | Purity | >85% (SDS-PAGE) |
| Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | P00480 |
|---|
Uniprot Id
P00480
Target Species
Human
Target Name
OTC
Target Full Name
Ornithine transcarbamylase, mitochondrial
Target Function
Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline. The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion.
Target Involvement
Ornithine carbamoyltransferase deficiency (OTCD)
Target Subcellular Location
Mitochondrion matrix.
Target Protein Families
ATCase/OTCase family
Target Tissue Specificity
Mainly expressed in liver and intestinal mucosa.
Target Synonyms
EC 2.1.3.3; MGC129967; MGC129968; MGC138856; OCTD; Ornithine Carbamoyltransferase; Ornithine carbamoyltransferase mitochondrial; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase
Target Background
This nuclear gene encodes a mitochondrial matrix enzyme. The encoded protein is involved in the urea cycle which functions to detoxify ammonia into urea for excretion. Mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia.
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