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Mouse Anti-OTC (AA 33-354) Recombinant Antibody (8A1) (CBMAB-O0680-CQ)

This product is a mouse antibody that recognizes OTC (AA 33-354). The antibody 8A1 can be used for immunoassay techniques such as: WB, IHC, IHC-P.
See all OTC antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
8A1
Antibody Isotype
IgG1
Application
WB, IHC, IHC-P

Basic Information

Immunogen
Recombinant protein fragment corresponding to amino acids 33-354 of human OTC (NP_000522) produced in E. coli
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 33-354

Target

Full Name
Ornithine Carbamoyltransferase
Introduction
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.
Entrez Gene ID
5009
UniProt ID
P00480
Alternative Names
Ornithine Carbamoyltransferase; Ornithine Transcarbamylase; EC 2.1.3.3; OTCase; Ornithine Carbamoyltransferase, Mitochondrial; EC 2.1.3; OCTD
Function
Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline (PubMed:6372096, PubMed:8112735, PubMed:2556444).
The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion (PubMed:2556444).
Biological Process
Ammonium homeostasisManual Assertion Based On ExperimentIMP:BHF-UCL
Anion homeostasisIEA:Ensembl
Arginine biosynthetic process via ornithineManual Assertion Based On ExperimentIBA:GO_Central
Citrulline biosynthetic processManual Assertion Based On ExperimentIDA:BHF-UCL
Liver developmentIEA:Ensembl
Midgut developmentIEA:Ensembl
Ornithine catabolic processManual Assertion Based On ExperimentIDA:BHF-UCL
Response to biotinIEA:Ensembl
Response to insulinIEA:Ensembl
Response to xenobiotic stimulusIEA:Ensembl
Response to zinc ionIEA:Ensembl
Urea cycleManual Assertion Based On ExperimentIDA:BHF-UCL
Cellular Location
Mitochondrion matrix
Involvement in disease
Ornithine carbamoyltransferase deficiency (OTCD):
An X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.
PTM
Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.

Sysák, R., Brennerová, K., Krlín, R., Štencl, P., Rusňák, I., & Vargová, M. (2022). Effect of ornithine transcarbamylase (OTC) deficiency on pregnancy and puerperium. Diagnostics, 12(2), 415.

Ozdemir, Y., Cag, M., Gul, S., Yüksel, Z., & Ergoren, M. C. (2022). In silico analysis of a de novo OTC variant as a cause of ornithine transcarbamylase deficiency. Applied Immunohistochemistry & Molecular Morphology, 30(2), 153-156.

Kido, J., Sugawara, K., Sawada, T., Matsumoto, S., & Nakamura, K. (2022). Pathogenic variants of ornithine transcarbamylase deficiency: Nation-wide study in Japan and literature review. Frontiers in Genetics, 13, 952467.

Lichter-Konecki, U., Caldovic, L., Morizono, H., Simpson, K., Mew, A., & MacLeod, E. (2022). Ornithine transcarbamylase deficiency.

Scharre, S., Posset, R., Garbade, S. F., Gleich, F., Seidl, M. J., Druck, A. C., ... & Zeman, J. (2022). Predicting the disease severity in male individuals with ornithine transcarbamylase deficiency. Annals of clinical and translational neurology, 9(11), 1715-1726.

Couchet, M., Breuillard, C., Corne, C., Rendu, J., Morio, B., Schlattner, U., & Moinard, C. (2021). Ornithine transcarbamylase–From structure to metabolism: An update. Frontiers in Physiology, 12, 748249.

Peng, M. Z., Li, X. Z., Mei, H. F., Sheng, H. Y., Yin, X., Jiang, M. Y., ... & Liu, L. (2020). Clinical and biochemical characteristics of patients with ornithine transcarbamylase deficiency. Clinical Biochemistry, 84, 63-72.

Hidaka, M., Higashi, E., Uwatoko, T., Uwatoko, K., Urashima, M., Takashima, H., ... & Sugimori, H. (2020). Late‐onset ornithine transcarbamylase deficiency: A rare cause of recurrent abnormal behavior in adults. Acute medicine & surgery, 7(1), e565.

Lu, D., Han, F., Qiu, W., Zhang, H., Ye, J., Liang, L., ... & Han, L. (2020). Clinical and molecular characteristics of 69 Chinese patients with ornithine transcarbamylase deficiency. Orphanet journal of rare diseases, 15, 1-13.

Pizzi, M. A., Alejos, D., Hasan, T. F., Atwal, P. S., Krishnaiengar, S. R., & Freeman, W. D. (2019). Adult presentation of ornithine transcarbamylase deficiency: 2 illustrative cases of phenotypic variability and literature review. The Neurohospitalist, 9(1), 30-36.

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For research use only. Not intended for any clinical use.

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