Mouse Anti-ADSL Recombinant Antibody (V2-180027) (CBMAB-A1429-YC)

Name: Mouse Anti-ADSL Recombinant Antibody (V2-180027)
SKU: CBMAB-A1429-YC
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

V2-180027

Application

WB, IHC-F, ELISA, ICC, IF

Immunogen

Recombinant human ADSL(1-484aa) purified from E. coli

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG1, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:1,000
IF(ICC)	1:100
FC	1:10-1:1,000

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

Buffer

PBS pH7.4, 10% glycerol

Preservative

0.02% sodium azide

Concentration

1 mg/ml

Storage

Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

Target

Full Name

Adenylosuccinate Lyase

Introduction

ADSL belongs to the lyase 1 family. It is an essential enzyme involved in purine metabolism, and catalyzes two non-sequential reactions in the de novo purine biosynthetic pathway: the conversion of succinylaminoimidazole carboxamide ribotide (SAICAR) to a

Entrez Gene ID

158

UniProt ID

P30566

Alternative Names

Adenylosuccinate Lyase; Adenylosuccinase; EC 4.3.2.2; ASASE; AMPS; ASL; EC 4.3.2;

Function

Catalyzes two non-sequential steps in de novo AMP synthesis: converts (S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamido)succinate (SAICAR) to fumarate plus 5-amino-1-(5-phospho-D-ribosyl)imidazole-4-carboxamide, and thereby also contributes to de novo IMP synthesis, and converts succinyladenosine monophosphate (SAMP) to AMP and fumarate.

Biological Process

de novo' AMP biosynthetic process
'de novo' IMP biosynthetic process
AMP biosynthetic process
Purine nucleotide biosynthetic process
Purine ribonucleoside monophosphate biosynthetic process

Cellular Location

Cytosol; Protein-containing complex

Involvement in disease

Adenylosuccinase deficiency (ADSLD): An autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present.

Taha-Mehlitz, S., Bianco, G., Coto-Llerena, M., Kancherla, V., Bantug, G. R., Gallon, J., ... & Piscuoglio, S. (2021). Adenylosuccinate lyase is oncogenic in colorectal cancer by causing mitochondrial dysfunction and independent activation of NRF2 and mTOR-MYC-axis. Theranostics, 11(9), 4011.

Mastrogiorgio, G., Macchiaiolo, M., Buonuomo, P. S., Bellacchio, E., Bordi, M., Vecchio, D., ... & Bartuli, A. (2021). Clinical and molecular characterization of patients with adenylosuccinate lyase deficiency. Orphanet journal of rare diseases, 16(1), 1-10.

Macchiaiolo, M., Buonuomo, P. S., Mastrogiorgio, G., Bordi, M., Testa, B., Weber, G., ... & Bartuli, A. (2020). Very mild isolated intellectual disability caused by adenylosuccinate lyase deficiency: a new phenotype. Molecular genetics and metabolism reports, 23, 100592.

Andelman-Gur, M. M., Saitsu, H., Matsumoto, N., Spiegel, R., Yosovich, K., Lev, D., ... & Blumkin, L. (2020). Myoclonic tremor status as a presenting symptom of adenylosuccinate lyase deficiency. European journal of medical genetics, 63(12), 104061.

Zurlo, G., Liu, X., Takada, M., Fan, C., Simon, J. M., Ptacek, T. S., ... & Zhang, Q. (2019). Prolyl hydroxylase substrate adenylosuccinate lyase is an oncogenic driver in triple negative breast cancer. Nature communications, 10(1), 1-15.

Oduselu, G. O., Ajani, O. O., Ajamma, Y. U., Brors, B., & Adebiyi, E. (2019). Homology modelling and molecular docking studies of selected substituted Benzo [d] imidazol-1-yl) methyl) benzimidamide scaffolds on Plasmodium falciparum adenylosuccinate lyase receptor. Bioinformatics and biology insights, 13, 1177932219865533.

Park, H., Ohshima, K., Nojima, S., Tahara, S., Kurashige, M., Hori, Y., ... & Morii, E. (2018). Adenylosuccinate lyase enhances aggressiveness of endometrial cancer by increasing killer cell lectin-like receptor C3 expression by fumarate. Laboratory Investigation, 98(4), 449-461.

Mao, X., Li, K., Tang, B., Luo, Y., Ding, D., Zhao, Y., ... & Guo, J. (2017). Novel mutations in ADSL for Adenylosuccinate Lyase deficiency identified by the combination of trio-WES and constantly updated guidelines. Scientific reports, 7(1), 1-7.

Macchiaiolo, M., Barresi, S., Cecconi, F., Zanni, G., Niceta, M., Bellacchio, E., ... & Bartuli, A. (2017). A mild form of adenylosuccinate lyase deficiency in absence of typical brain MRI features diagnosed by whole exome sequencing. Italian journal of pediatrics, 43(1), 1-7.

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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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Isotype control

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

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