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Mouse Anti-CPS1 Recombinant Antibody (CBXC-3108) (V2LY-1206-LY982)


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Published Data
V2LY-1206-LY982-1
Mouse Anti-CPS1 Recombinant Antibody (CBXC-3108) (V2LY-1206-LY982) in IHC
Represents immunohistochemistry findings in human liver biopsies. ...View More
Gallego-Durán, R., Ampuero, J., Pastor-Ramírez, H., Álvarez-Amor, L., Del Campo, J. A., Maya-Miles, D., ... & Romero-Gómez, M. (2022). Liver injury in non-alcoholic fatty liver disease is associated with urea cycle enzyme dysregulation. Scientific Reports, 12(1), 3418.
Distributed under Open Access license CC BY 4.0, without modification.
V2LY-1206-LY982-2
Mouse Anti-CPS1 Recombinant Antibody (CBXC-3108) (V2LY-1206-LY982) in IHC
CPS1 findings in CDA-HFD animals. (A) represents the number of positive pixels quantified by ImageJ and (B) shows the staining. ...View More
Gallego-Durán, R., Ampuero, J., Pastor-Ramírez, H., Álvarez-Amor, L., Del Campo, J. A., Maya-Miles, D., ... & Romero-Gómez, M. (2022). Liver injury in non-alcoholic fatty liver disease is associated with urea cycle enzyme dysregulation. Scientific Reports, 12(1), 3418.
Distributed under Open Access license CC BY 4.0, without modification.
V2LY-1206-LY982-3
Mouse Anti-CPS1 Recombinant Antibody (CBXC-3108) (V2LY-1206-LY982) in WB
Comparison of the expression of selected proteins in cytosolic (CYT) and high-purity mitochondrial (MIT) fractions isolated from MCF7 cells using a QProteome Mitochondria Isolation Kit. The level of tested proteins was assessed using western blot analysis employing relevant antibodies. ...View More
Daniel, P., Halada, P., Jelínek, M., Balušíková, K., & Kovář, J. (2019). Differentially expressed mitochondrial proteins in human MCF7 breast cancer cells resistant to paclitaxel. International journal of molecular sciences, 20(12), 2986.
Distributed under Open Access license CC BY 4.0, without modification.
V2LY-1206-LY982-4
Mouse Anti-CPS1 Recombinant Antibody (CBXC-3108) (V2LY-1206-LY982) in IF
Cellular distribution of CPS1 (carbamoyl-phosphate synthetase 1) in paclitaxel-sensitive MCF7 cells and paclitaxel-resistant MCF7/PacR cells. The localization of CPS1 was detected using confocal microscopy. The localization of CPS1 (green), mitochondria (red), nuclei (blue) and the merge are shown. The data shown were obtained in one representative experiment of two independent experiments. ...View More
Daniel, P., Halada, P., Jelínek, M., Balušíková, K., & Kovář, J. (2019). Differentially expressed mitochondrial proteins in human MCF7 breast cancer cells resistant to paclitaxel. International journal of molecular sciences, 20(12), 2986.
Distributed under Open Access license CC BY 4.0, without modification.
V2LY-1206-LY982-5
Mouse Anti-CPS1 Recombinant Antibody (CBXC-3108) (V2LY-1206-LY982) in WB
Western blot analysis demonstrates a SIRT5-dependent increase in deacetylation of CPS1 with hydralazine treatment. 5 µM resveratrol used as a positive control (n = 2-3, mean ± SD). ...View More
Dehghan, E., Goodarzi, M., Saremi, B., Lin, R., & Mirzaei, H. (2019). Hydralazine targets cAMP-dependent protein kinase leading to sirtuin1/5 activation and lifespan extension in C. elegans. Nature communications, 10(1), 4905.
Distributed under Open Access license CC BY 4.0, without modification.

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
CBXC-3108
Antibody Isotype
IgG1, κ
Application
WB, IP, IF, ELISA, IHC-P

Basic Information

Immunogen
Amino acids 1361-1500 mapping at the C-terminus of human CPS1.
Host Species
Mouse
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG1, κ
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:100-1:1,000
IP1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)1:50-1:500
ELISA1:100-1:1,000
IHC-P1:50-1:500

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

Format
Liquid
Buffer
0.1% gelatin
Preservative
0.09% sodium azide
Concentration
0.2 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Carbamoyl-Phosphate Synthase 1
Entrez Gene ID
Human1373
Mouse227231
Rat497840
UniProt ID
HumanP31327
MouseQ8C196
RatP07756
Function
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Biological Process
de novo' pyrimidine nucleobase biosynthetic process Source: InterPro
Anion homeostasis Source: Ensembl
Carbamoyl phosphate biosynthetic process Source: UniProtKB
Cellular response to ammonium ion Source: BHF-UCL
Cellular response to cAMP Source: Ensembl
Cellular response to fibroblast growth factor stimulus Source: Ensembl
Cellular response to glucagon stimulus Source: Ensembl
Cellular response to oleic acid Source: Ensembl
Citrulline biosynthetic process Source: BHF-UCL
Glutamine metabolic process Source: GO_Central
Hepatocyte differentiation Source: Ensembl
Homocysteine metabolic process Source: UniProtKB
Midgut development Source: Ensembl
Nitric oxide metabolic process Source: BHF-UCL
Nitrogen compound metabolic process Source: GO_Central
Response to amine Source: Ensembl
Response to amino acid Source: Ensembl
Response to dexamethasone Source: Ensembl
Response to drug Source: Ensembl
Response to food Source: Ensembl
Response to growth hormone Source: Ensembl
Response to lipopolysaccharide Source: UniProtKB
Response to starvation Source: Ensembl
Response to toxic substance Source: Ensembl
Response to zinc ion Source: Ensembl
Triglyceride catabolic process Source: BHF-UCL
Urea cycle Source: Reactome
Vasodilation Source: BHF-UCL
Cellular Location
Nucleolus; Mitochondrion
Involvement in disease
Carbamoyl phosphate synthetase 1 deficiency (CPS1D):
An autosomal recessive disorder of the urea cycle causing hyperammonemia. It can present as a devastating metabolic disease dominated by severe hyperammonemia in neonates or as a more insidious late-onset condition, generally manifesting as life-threatening hyperammonemic crises under catabolic situations. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation.
Pulmonary hypertension, neonatal (PHN):
Disease susceptibility is associated with variants affecting the gene represented in this entry. CPS1 variants influence the availability of precursors for nitric oxide (NO) synthesis and play a role in clinical situations where endogenous NO production is critically important, such as neonatal pulmonary hypertension, increased pulmonary artery pressure following surgical repair of congenital heart defects or hepatovenocclusive disease following bone marrow transplantation. Infants with neonatal pulmonary hypertension homozygous for Thr-1406 have lower L-arginine concentrations than neonates homozygous for Asn-1406 (PubMed:11407344). A disease characterized by elevated pulmonary artery pressure. Pulmonary hypertension in the neonate is associated with multiple underlying problems such as respiratory distress syndrome, meconium aspiration syndrome, congenital diaphragmatic hernia, bronchopulmonary dysplasia, sepsis, or congenital heart disease.
PTM
Undergoes proteolytic cleavage in the C-terminal region corresponding to the loss of approximately 12 AA residues from the C-terminus.
Succinylated at Lys-287 and Lys-1291. Desuccinylated at Lys-1291 by SIRT5, leading to activation (By similarity).
Glutarylated. Glutarylation levels increase during fasting. Deglutarylated by SIRT5 at Lys-55, Lys-219, Lys-412, Lys-889, Lys-892, Lys-915, Lys-1360 and Lys-1486, leading to activation.
More Infomation

Choi, Y., Oh, A., Lee, Y., Kim, G. H., Choi, J. H., Yoo, H. W., & Lee, B. H. (2022). Unfavorable clinical outcomes in patients with carbamoyl phosphate synthetase 1 deficiency. Clinica Chimica Acta, 526, 55-61.

Green, S. R., & Storey, K. B. (2019). Purification of carbamoyl phosphate synthetase 1 (CPS1) from wood frog (Rana sylvatica) liver and its regulation in response to ice-nucleation and subsequent whole-body freezing. Molecular and Cellular Biochemistry, 455(1), 29-39.

Liu, X., Zhang, X., Bi, J., Li, Z., Zhang, Z., & Kong, C. (2019). Caspase recruitment domain family member 10 regulates carbamoyl phosphate synthase 1 and promotes cancer growth in bladder cancer cells. Journal of cellular and molecular medicine, 23(12), 8128-8138.

Yap, S., Gougeard, N., Hart, A. R., Barcelona, B., & Rubio, V. (2019). N‐carbamoylglutamate‐responsive carbamoyl phosphate synthetase 1 (CPS1) deficiency: A patient with a novel CPS1 mutation and an experimental study on the mutation's effects. JIMD reports, 48(1), 36-44.

Khoja, S., Nitzahn, M., Truong, B., Lambert, J., Willis, B., Allegri, G., ... & Lipshutz, G. S. (2019). A constitutive knockout of murine carbamoyl phosphate synthetase 1 results in death with marked hyperglutaminemia and hyperammonemia. Journal of inherited metabolic disease, 42(6), 1044-1053.

El‐Sheikh, R. M., Mansy, S. S., Nessim, I. G., Hosni, H. N., El Hindawi, A., Hassanein, M. H., & AbdelFattah, A. S. (2019). Carbamoyl phosphate synthetase 1 (CPS1) as a prognostic marker in chronic hepatitis C infection. Apmis, 127(2), 93-105.

Choi, R., Park, H. D., Yang, M., Ki, C. S., Lee, S. Y., Kim, J. W., ... & Park, W. S. (2017). Novel pathogenic variant (c. 580C> T) in the CPS1 gene in a newborn with carbamoyl phosphate synthetase 1 deficiency identified by whole exome sequencing. Annals of laboratory medicine, 37(1), 58-62.

Diez-Fernandez, C., & Häberle, J. (2017). Targeting CPS1 in the treatment of Carbamoyl phosphate synthetase 1 (CPS1) deficiency, a urea cycle disorder. Expert Opinion on Therapeutic Targets, 21(4), 391-399.

Chen, Z., Tang, N., Wang, X., & Chen, Y. (2017). The activity of the carbamoyl phosphate synthase 1 promoter in human liver‐derived cells is dependent on hepatocyte nuclear factor 3‐beta. Journal of cellular and molecular medicine, 21(9), 2036-2045.

Yang, X., Shi, J., Lei, H., Xia, B., & Mu, D. (2017). Neonatal-onset carbamoyl phosphate synthetase I deficiency: A case report. Medicine, 96(26).

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

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