Product Details

Uniprot No.

P00966

Target Names

ASS1

Alternative Names

Argininosuccinate synthase (EC 6.3.4.5) (Citrulline--aspartate ligase), ASS1, ASS

Species Reactivity

Human

Immunogen

A synthesized peptide derived from Human ASS1

Immunogen Species

Homo sapiens (Human)

Conjugate

Non-conjugated

Clonality

Monoclonal

Isotype

Rabbit IgG

Clone No.

18G11

Purification Method

Affinity-chromatography

Concentration

It differs from different batches. Please contact us to confirm it.

Buffer

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.

Form

Liquid

Tested Applications

ELISA, IHC, IF, FC

Recommended Dilution

Application	Recommended Dilution
IHC	1:50-1:200
IF	1:50-1:200
FC	1:50-1:200

Protocols

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
Flow Cytometry (FC) Protocol

Troubleshooting and FAQs

Antibody FAQs

Storage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Lead Time

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Description

Argininosuccinate synthase 1 (ASS1) catalyzes a critical step in the urea cycle, converting citrulline and aspartate to argininosuccinate. Beyond its metabolic function, ASS1 has emerged as a significant focus in cancer research, where its expression status influences tumor arginine auxotrophy and therapeutic vulnerability. Understanding ASS1 expression patterns provides valuable insights into metabolic reprogramming in disease states and potential intervention strategies.

This recombinant monoclonal antibody, generated against a synthetic peptide derived from human ASS1, offers the reproducibility and consistency that demanding research applications require. As a sequence-defined recombinant clone (18G11), it eliminates the lot-to-lot variability inherent in traditional hybridoma-derived antibodies, ensuring your experimental conditions remain stable across extended studies and collaborative projects.

Validation across multiple detection platforms demonstrates this antibody's versatility in your workflow. Immunohistochemistry performed on paraffin-embedded human kidney tissue using a Leica Bond system with citrate buffer antigen retrieval shows reliable detection at 1:100 dilution. For cellular studies, immunofluorescence staining in HeLa cells reveals clear signal at 1:50 dilution, while flow cytometry analysis of Jurkat cells demonstrates a distinct positive shift compared to isotype control, confirming specific intracellular detection of ASS1.

The antibody is supplied in a glycerol-containing buffer optimized for long-term stability at -20°C or -80°C, with affinity chromatography purification ensuring high purity for sensitive applications. Whether investigating urea cycle disorders, characterizing metabolic phenotypes in cancer models, or exploring ASS1's role in cellular signaling pathways, this antibody provides a dependable tool for advancing your research in metabolism and oncology.

Usage

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

Target Background

Function

One of the enzymes of the urea cycle, the metabolic pathway transforming neurotoxic amonia produced by protein catabolism into inocuous urea in the liver of ureotelic animals. Catalyzes the formation of arginosuccinate from aspartate, citrulline and ATP and together with ASL it is responsible for the biosynthesis of arginine in most body tissues.

Gene References into Functions

In ASS1-knockout cells, DEPTOR, an inhibitor of mTORC1 signal, was downregulated and mTORC1 signaling was more activated in response to arginine. PMID: 28358054
Results indicate that the reduced ASS1 expression in Dox-resistant sarcomas may contribute to drug resistance in association with the expression of P-glycoprotein. PMID: 27683125
Cisplatin-induced synthetic lethality to arginine-starvation therapy by transcriptional suppression of ASS1 is regulated by DEC1, HIF-1alpha, and c-Myc transcription network and is independent of ASS1 promoter DNA methylation. PMID: 27765932
Low expression of ASS1 is associated with glioblastoma. PMID: 27431689
defined a new hepatocellular adenoma subgroup at a high risk of bleeding PMID: 28646562
ASS1 acetylation by CLOCK exhibits circadian oscillation in human cells and mouse liver, possibly caused by rhythmic interaction between CLOCK and ASS1, leading to the circadian regulation of ASS1 and ureagenesis. PMID: 28985504
update reports 137 mutations in the ASS1 gene (64 of which are novel), consisting of 89 missense mutations, 19 nonsense mutations, 17 mutations that affect splicing, and 12 deletions; the change p.Gly390Arg is by far the most common mutation and is widely spread throughout the world PMID: 28111830
Of 21 ASS potential kinetic mutations, 13 were totally inactive while 8 exhibited decreased affinity for aspartate and citrulline. PMID: 27287393
Low ASS1 expression was associated with higher recurrence , shorter disease-free survival and shorter overall survival in patients with pancreatic ductal adenocarcinoma. PMID: 28187218
The authors showed that ASS1 mutations linked to type I citrullinemia disrupt the ASS1-PRMT7 interaction, which might explain the molecular pathogenesis of the disease. PMID: 28587924
Identification of three novel CTLN1 mutations in fourteen patients with citrullinemia type 1 has been reported. PMID: 28132756
In this trial, arginine deprivation with ADI-PEG20 improved PFS in patients with ASS1-deficient mesothelioma. Targeting arginine is safe and warrants further clinical investigation in arginine-dependent cancers. PMID: 27584578
combining hypoxia and ADI-PEG20 synergistically inhibited ASS1. PMID: 26972697
ASS1 genomic variants (rs10901080 and rs10793902) can serve as pharmacogenomic biomarkers to predict hydroxyurea treatment efficacy in sickle cell disease/beta-thalassemia compound heterozygous patients. PMID: 26895070
results show that ASS1 is elevated at the mRNA and protein levels in mesothelioma 3D spheroids and in human pleural mesotheliomas. We also have uncovered a survival role for ASS1 PMID: 26982031
results demonstrate that ASS1 downregulation is a novel mechanism supporting cancerous proliferation, and they provide a metabolic link between the urea cycle enzymes and pyrimidine synthesis PMID: 26560030
More than 50 % of the high-grade pulmonary neuroendocrine carcinomas tested lack immunohistochemically detectable ASS, suggesting that they are auxotrophic for arginine and potential candidates for arginine deprivation therapy. PMID: 25548129
Three novel splicing and missense mutations have been identified in the ASS1 gene in classical citrullinemia patients. PMID: 25179242
ASS expression in gastric cancer was associated with a poor prognosis. PMID: 25333458
we reviewed the English literature on mutations in the ASS and SLC25A13 genes, and their genotype-phenotype correlations to provide valuable insights into the molecular genetic background of citrullinemia--{REVIEW} PMID: 24508627
Data indicate that argininosuccinate synthetase 1 (ASS1) abundance is a prognostic factor for overall breast cancer survival. PMID: 24692592
Argininosuccinate synthetase gene is silenced by CpG methylation in children with phenylketonuria.The promoter of argininosuccinate synthetase was methylated which silence the transcription of argininosuccinate synthetase. PMID: 24192130
Aberrant loss of ASS1 strongly links to DNA methylation in neck nodal metastases of NPC and significantly correlates with advanced T classification, and independently predicts worse DSS and DMFS in independent NPC tissue specimens. PMID: 23897555
Together with the observation that AS1 protein and mRNA levels decrease during wild-type infection, this work suggests that reduced AS1 activity is partially responsible for the metabolic program induced by infection. PMID: 24297925
Our findings highlight ASS1 as a novel tumor suppressor in myxofibrosarcomas, with loss of expression linked to promoter methylation, clinical aggressiveness, and sensitivity to ADI-PEG20. PMID: 23549872
ASS expression is decreased significantly in hepatocellular carcinoma tissues. PMID: 23339388
Mutations are identified only in exons of ASS1 gene from the Korean patients with citrullinemia type I. PMID: 23099195
The present study demonstrated a key regulatory role of KLF4 in the endothelial ASS1 expression and NO production in response to laminar shear stress. PMID: 22430140
The ASS release represents a potential counteracting liver reaction to LPS. PMID: 21481813
the expression of ASS1 harmonized with that of NOS3 may be important for the optimized endothelial NO production and the prevention of the inflammatory monocyte adhesion to endothelial cells. PMID: 21106532
Analysis of five SNPs of the ASS1 gene revealed that the G allele of rs7860909 is associated with increased CL/P risk. PMID: 20739017
In patients with osteosarcoma, reduced expression of ASS is not only a novel predictive biomarker for the development of metastasis, but also a potential target for pharmacologic intervention. PMID: 20159990
Studies indicate that the proximal region of the AS promoter contains an E-box that is recognized by c-Myc and HIF-1alpha and a GC-box by Sp4. PMID: 19934275
Mutational analysis revealed three alleles with a common mutation and five new mutations. PMID: 11708871
structure and use in diagnosing citrullinemia PMID: 11941481
Sixteen novel mutations have been identified in the argininosuccinate synthetase gene in citrullinemia patients. PMID: 12815590
argininosuccinate synthetase gene expression is stimulated by glutamine through cytosolic O-glycosylation of Sp1 in tumor cells PMID: 14570901
Argininosuccinate synthetase has a role in preventing autotoxicity from nitric oxide overproduction PMID: 15192091
Extensive mutation study by direct genomic sequencing of ASS demonstrated a homozygous G117S mutation in one patient and homozygous R363W mutations in the other two families PMID: 16124451
IL-1beta induces nitric oxide which has antagonistic effects on argininosuccinate synthetase gene and on the activity of argininosuccinate synthetase PMID: 16380201
ASS, the c-myc-regulated gene is involved in genotype-C-HBV-related HCC, suggesting that c-myc is related to the hepatocarcinogenic activity of genotype-C HBV. PMID: 16703398
Low argininosuccinate synthetase is associated with renal cell carcinoma PMID: 17096330
high levels of AS expression, which may be required for several arginine-dependent processes in cancer, including the production of nitric oxide, proline, pyrimidines and polyamines, is regulated by TNF-alpha PMID: 17354225
HSCARG regulation of argininosuccinate synthetase activity is crucial for maintaining the intracellular balance between redox state and nitric oxide levels PMID: 18263583
These results show that liver-specific enhancement of ASS gene expression is mediated in part by the cAMP signaling pathway through a distal CRE site. PMID: 18840401
argininosuccinate synthetase behaves as a typical suckling enzyme because its expression all but disappears in the putative weaning period of human infants. PMID: 19000307
a survey of the correlation between mutations in the ASS1 gene and the respective clinical courses of citrullinemia type I as described so far (Review) PMID: 19006241
The studied families showed the same mutation: ASS~p.G390R, associated with the early-onset/severe phenotype. PMID: 19358837
changes in gene expression are induced by laminar shear stress as well as by cellular senescence PMID: 19409979
Epigenetic silencing of argininosuccinate synthetase confers resistance to platinum-induced cell death but collateral sensitivity to arginine auxotrophy in ovarian cancer. PMID: 19533750

Hide All

Involvement in disease

Citrullinemia 1 (CTLN1)

Subcellular Location

Cytoplasm, cytosol.

Protein Families

Argininosuccinate synthase family, Type 1 subfamily

Tissue Specificity

Expressed in adult liver.

Database Links

HGNC: 758

OMIM: 215700

KEGG: hsa:445

STRING: 9606.ENSP00000253004

UniGene: Hs.160786

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Code	CSB-RA095825A0HU
Size	US$210
	Order now
Image	IHC image of CSB-RA095825A0HU diluted at 1:100 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.56% DAB. Immunofluorescence staining of Hela with CSB-RA095825A0HU at 1:50, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 516-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). Overlay Peak curve showing Jurkat cells stained with CSB-RA095825A0HU (red line) at 1:50. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1µg/110⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated Goat Anti-rabbit IgG(H+L) at 1:200 dilution for 35min at 4℃.Control antibody (green line) was rabbit IgG (1µg/110⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
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ASS1 Recombinant Monoclonal Antibody

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Target Background