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


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Summary

Host Animal
Rabbit
Specificity
Human, Mouse, Rat
Clone
A1989
Antibody Isotype
IgG
Application
WB, IHC-P, FC

Basic Information

Immunogen
Synthetic peptide within human CTPS aa 250-300.
Host Species
Rabbit
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:500-1:1,000
IHC-P1:50
FC1:500-1:1,000

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

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

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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