Rabbit Anti-CTPS1 Recombinant Antibody (A1989) (CBMAB-A1989-LY)

Name: Rabbit Anti-CTPS1 Recombinant Antibody (A1989)
SKU: CBMAB-A1989-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Rabbit

Clone

A1989

Application

WB, IHC-P, FC

Immunogen

Synthetic peptide within human CTPS aa 250-300.

Host Species

Rabbit

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG

Clonality

Monoclonal Antibody

Application Notes

Application	Note
WB	1:500-1:1,000
IHC-P	1:50
FC	1:500-1:1,000

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

Format

Liquid

Buffer

TBS, pH 7.4, 40% glycerol, 0.05% BSA

Preservative

0.05% sodium azide

Concentration

1 mg/ml

More Infomation

Target

Full Name

CTP Synthase 1

Entrez Gene ID

1503

UniProt ID

P17812

Function

This enzyme is involved in the de novo synthesis of CTP, a precursor of DNA, RNA and phospholipids. Catalyzes the ATP-dependent amination of UTP to CTP with either L-glutamine or ammonia as a source of nitrogen. This enzyme and its product, CTP, play a crucial role in the proliferation of activated lymphocytes and therefore in immunity.

Biological Process

de novo' CTP biosynthetic process Source: UniProtKB-UniPathway
B cell proliferation Source: UniProtKB
CTP biosynthetic process Source: UniProtKB
Glutamine metabolic process Source: UniProtKB-KW
Nucleobase-containing compound metabolic process Source: ProtInc
Nucleobase-containing small molecule interconversion Source: Reactome
Pyrimidine nucleobase biosynthetic process Source: GO_Central
Response to drug Source: ProtInc
T cell proliferation Source: UniProtKB

Cellular Location

Cytosol. Mainly cytosolic but when active detected in long filamentous structures (PubMed:25223282). Co-localizes with TNK2 in the cytosolic filaments (By similarity).

Involvement in disease

Immunodeficiency 24 (IMD24):
The disease is caused by variants affecting the gene represented in this entry. A unique and recessive G to C mutation probably affecting a splice donor site at the junction of intron 17-18 and exon 18 has been identified in all patients. It results in expression of an abnormal transcript lacking exon 18 and a complete loss of the expression of the protein.
A life-threatening immunodeficiency, characterized by an impaired capacity of activated T and B cells to proliferate in response to antigen receptor-mediated activation. Patients have early onset of severe chronic viral infections, mostly caused by herpes viruses, including EBV and varicella zooster virus (VZV), and also suffer from recurrent encapsulated bacterial infections, a spectrum of infections typical of a combined deficiency of adaptive immunity.

Pfeiffer, C., Bolomsky, A., Zojer, N., Schreder, M., Asnagli, H., Parker, A., & Ludwig, H. (2021). P-096: Tackling pyrimidine biosynthesis-CTP Synthase 1 is a novel target in the treatment of multiple myeloma. Clinical Lymphoma Myeloma and Leukemia, 21, S91-S92.

Rao, Y., Wang, T. Y., Qin, C., Espinosa, B., Liu, Q., Ekanayake, A., ... & Feng, P. (2021). Targeting CTP synthetase 1 to restore interferon induction and impede nucleotide synthesis in SARS-CoV-2 infection. bioRxiv.

Parker, A. (2020). STP938, a Novel, Potent and Selective Inhibitor of CTP Synthase 1 (CTPS1) Is a Targeted Therapy Specifically Blocking De Novo Nucleotide Synthesis in Lymphomas and Leukemias. Blood, 136, 31.

Martin, E., Minet, N., Boschat, A. C., Sanquer, S., Sobrino, S., Lenoir, C., ... & Genomics England Research Consortium. (2020). Impaired lymphocyte function and differentiation in CTPS1-deficient patients result from a hypomorphic homozygous mutation. JCI insight, 5(5).

Nademi, Z., Wynn, R. F., Slatter, M., Hughes, S. M., Bonney, D., Qasim, W., ... & Arkwright, P. D. (2019). Hematopoietic stem cell transplantation for cytidine triphosphate synthase 1 (CTPS1) deficiency. Bone marrow transplantation, 54(1), 130-133.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

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

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