CPS1

What is CPS1 Protein?

CPS1 protein, short for carbamoyl phosphate synthetase 1, is like a hardworking enzyme that hangs out in our liver's mitochondria. It kicks off the urea cycle, which is kind of like the body's way of getting rid of excess nitrogen. Think of it as helping to clean out those byproducts we don't need from breaking down proteins. When CPS1 isn't working right, it can lead to a build-up of toxic stuff in the blood—not great for health. Interestingly, some research is looking at how CPS1 behaves in various conditions, including certain cancers, because it might be overactive or totally missing in those disease states.

What is the Function of CPS1 Protein?

The CPS1 protein is basically a key player in getting rid of extra nitrogen in our body. It kicks off the urea cycle, which helps convert this nitrogen into urea. Then, our body can get rid of it through urine. Without CPS1 doing its thing, ammonia and other nasty stuff could build up, causing big health problems. So, in a way, CPS1 is like the behind-the-scenes hero of our body's clean-up crew.

CPS1 Related Signaling Pathway

The CPS1-related signaling pathway is all about how CPS1 fits into the broader network of reactions in the body. CPS1 starts off the urea cycle, which is crucial for turning toxic ammonia into urea so our body can safely expel it. But the story doesn't stop there. This pathway ties into energy production and metabolic regulation because it's happening in the mitochondria, the cell's powerhouses. Plus, changes in CPS1 activity are often linked with various diseases, including liver disorders and some cancers, because if this pathway gets disrupted, it can lead to an imbalance in nitrogen, causing a whole cascade of issues. So, while CPS1 is doing its specific job, it's also part of a bigger picture, influencing how our bodies manage waste and energy.

CPS1 Related Diseases

CPS1 is a crucial enzyme, and when it doesn't do its job right, it can cause significant health problems. One big issue is CPS1 deficiency, a genetic disorder where the enzyme falls short, leading to ammonia building up to toxic levels in the blood. This can result in symptoms like extreme tiredness, vomiting, and if left unchecked, serious neurological issues. Besides genetic issues, changes in CPS1 activity have been observed in liver diseases and certain types of cancer. In some cancers, like liver cancer, CPS1 might be found in unusual amounts, pointing to its role in disease progression. So, while CPS1 usually helps keep things running smoothly by managing nitrogen waste, when it goes off track, it can be linked to a range of health problems.

Bioapplications of CPS1

CPS1, or carbamoyl phosphate synthetase 1, isn't just about keeping our bodies toxin-free; it has some cool applications in the biotech and medical fields too. Because CPS1 is central to the urea cycle, it's a prime target for studying metabolic disorders. Researchers are looking at how tweaking CPS1 could help treat conditions where ammonia builds up, offering new ways to manage or even cure urea cycle disorders. In cancer research, scientists are exploring CPS1's role in tumor metabolism. By understanding CPS1 activity in cancer cells, new treatments might be developed to cut off the tumor's energy supply. On the diagnostics front, changes in CPS1 levels could serve as biomarkers for certain diseases, helping doctors catch some conditions earlier. So, CPS1 is not only vital for basic metabolism but also holds promise for advances in medicine and biotechnology.

Case Study 1: Liu H. et al. Am J Pathol. 2011

Carbamoyl phosphate synthetase 1 (CPS1) is an enzyme found in the liver, playing a critical role in the urea cycle. Researchers have noticed that CPS1 levels drop in liver cancer tissues and cells. This study dives into what's happening with the CPS1 gene in liver cancer. In liver cancer cells, CPS1 isn't expressed, while healthy liver cells have plenty of it. They found that using a demethylation agent like 5-azacytidine can restore CPS1 levels in these cancer cells. The tricky part is the methylation of two specific CpG sites near the gene's start point, which becomes hypermethylated in liver cancer. This hypermethylation shuts down CPS1, and could be a useful marker for identifying liver cancer.

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  Fig1. Western blot analysis of cellular extracts from cultured cells and patient tissues using Hep Par 1 antibody.
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  Fig2. Western blot analysis of samples prepared from LH86-pTOPO control cells and from LH86-pTOPO CPS1 cells using antibodies specific against CPS1 or Hep Par 1.

Case Study 2: Çeliktas M. et al. J Natl Cancer Inst. 2017

Liver kinase B1 (LKB1) acts as a tumor suppressor in lung adenocarcinoma (LADC). In 45 LADC cell lines, those without active LKB1 showed high levels of carbamoyl phosphate synthetase 1 (CPS1). Researchers found that lowering CPS1 slowed cell growth and enhanced the effect of certain drugs. Tissue analysis revealed CPS1 in 65.7% of LADC cases without LKB1, compared to just 5% with it. High CPS1 correlated with poorer survival rates in patients.

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  Fig3. Immunoblot analysis of LKB1 and CPS1 after overexpression of LKB1 in H1437 and H1944 cell lines.
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  Fig4. Knockdown of CPS1 with siRNA was confirmed by immunoblotting.

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  Fig1. Diagram of the mammalian urea cycle. (Matthew Nitzahn, 2020)
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  Fig2. Interactions regulating the gene and protein expression of CPS1. (Matthew Nitzahn, 2020)

CPS1 has several biochemical functions, for example, ATP binding,calcium ion binding,carbamoyl-phosphate synthase (ammonia) activity. Some of the functions are cooperated with other proteins, some of the functions could acted by CPS1 itself. We selected most functions CPS1 had, and list some proteins which have the same functions with CPS1. You can find most of the proteins on our site.

Function	Related Protein
protein binding	ABCF3,FHOD1,KLK5,KPNA5,AGFG1,IL1A,FAM188A,IRS1,KCNB1,MASP1
protein complex binding	TRAF2,SLC25A3,ERCC6,INS2,CAPG,LAMB3,SH2B3,RARB,RIPK1,UQCRC1
nucleotide binding	GSK3AB,ABCG4B,DNM2A,GMPPAA,PTK2BB,HIPK3A,NELFE,RBM23,KIF15,CAMK1DB
calcium ion binding	SLC24A4,CLSTN1,TPT1,HMCN1,TTYH1,PCDH2AB5,LDLRA,DCHS1,ARSA,FBN2B
modified amino acid binding	NOX4,MUT,DPEP1,MTHFR,CBS
carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity	CAD
ATP binding	MAP3K6,ATP1A1A.4,PDK4,GSK3AA,CENPE,IFIH1,PAK2B,TESK2,ACSL1,UBA1
endopeptidase activity	KLK1B3,Ren1,PSMB10,KLK1B1,MMP1,RHBDD1,NCSTN,APH1C,PSMA6,KLK1B4
ligase activity	UBE2KB,TTLL9,MGRN1,RNF123,RNF114,ATG10,TRIM32,CTPS1A,ARIH1,RNF43

CPS1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with CPS1 here. Most of them are supplied by our site. Hope this information will be useful for your research of CPS1.

Sirt5;Amy1;Ywhae;Pgls;Hsd3b7