Cofactors pterins. Medical search. Frequent questions

Molybdopterins are a class of cofactors found in most molybdenum-containing and all tungsten-containing enzymes. (wikipedia.org)
Enzymes that contain the molybdopterin cofactor include xanthine oxidase, DMSO reductase, sulfite oxidase, and nitrate reductase. (wikipedia.org)
In this case, the tungsten-selenium pair has been speculated to function analogously to the molybdenum-sulfur pairing of some molybdenum cofactor-requiring enzymes. (wikipedia.org)
Enzymes that use molybdopterin as cofactor or prosthetic group are given below. (wikipedia.org)
Molybdenum cofactor is associated with the enzymes sulfite oxidase, xanthine dehydrogenase, and aldehyde oxidase (see the image below). (medscape.com)
[ 2 ] The enzyme sulfite oxidase depends on the molybdenum-containing pterin cofactor, as do the enzymes xanthine dehydrogenase and aldehyde oxidase. (medscape.com)
c) Organic syntheses of heterocyclic compounds including cofactors, substrates, inhibitors and activators of the above enzymes, as well as relevant detergents and related lipids. (edu.au)
In these cases, cofactors with special chemical properties provide the necessary chemical expertise and enzymes use them as tiny tools to perform their reactions. (rcsb.org)
For instance, tetrahydrobiopterin is a cofactor used by several enzymes that juggle molecular oxygen, attaching it to amino acids and other molecules. (rcsb.org)
Two enzymes are then needed to strip off this unwanted oxygen and restore the cofactor. (rcsb.org)
Two enzymes regenerate tetrahydrobiopterin after it is used as a cofactor. (rcsb.org)
PTPS has an important role in the synthesis of BH4, which is an essential cofactor for a group of enzymes including phenylalanine hydroxylase, tyrosine and tryptophan hydroxylases. (rarediseasesjournal.com)
Inborn errors of metabolism form a large group of genetic diseases involving defects in genes coding for enzymes, receptors, cofactors etc. in metabolic pathways [ 1 ]. (alliedacademies.org)

To confirm that the selected candidate genes were regulated by dietary Mg(2+) availability, the expression levels of solute carrier family 41, member 3 (Slc41a3), pterin-4 α-carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor-1α (Pcbd1), TBC1 domain family, member 4 (Tbc1d4), and uromodulin (Umod) were determined by RT-PCR analysis. (nih.gov)
and (5) pterin-4α-carbinolamine dehydratase. (thieme-connect.de)
The first, pterin-4a-carbinolamine dehydratase (PCD, PDB entry 1dcp ), strips off the oxygen and releases it as a water molecule. (rcsb.org)

Molybdenum cofactor deficiency, a genetic illness. (wikipedia.org)
Molybdenum cofactor deficiency, which affects the functioning of sulfite oxidase, leads to a similar phenotype. (medscape.com)
However, many cases of this disorder are associated with deficiency of the molybdenum-containing pterin cofactor (molybdenum cofactor deficiency). (medscape.com)
Molybdenum cofactor deficiency. (medscape.com)
As sulfite oxidase deficiency and molybdenum cofactor deficiency have virtually identical phenotypes, the CNS toxicity appears to be secondary to loss of function of sulfite oxidase. (medscape.com)
Sulfite oxidase deficiency and molybdenum cofactor deficiency in the metabolism of sulfated amino acids. (medscape.com)
Closer to 100 cases of molybdenum cofactor deficiency have been reported. (medscape.com)
The estimated prevalence of molybdenum cofactor deficiency 1 per 100,000-200,000 newborns worldwide. (medscape.com)
Of note, some types of molybdenum cofactor deficiency have been responsive to treatment, leading to better longer-term outcomes. (medscape.com)
Isoxanthopterin is very low in some cases of hereditary xanthinuria (OMIM 278300 ) and molybdenum cofactor deficiency (OMIM 252150 ). (np-mrd.org)

Tetrahydrobiopterin loading test in xanthine dehydrogenase and molybdenum cofactor deficiencies. (wikigenes.org)

In enzymology, a pterin deaminase (EC 3.5.4.11) is an enzyme that catalyzes the chemical reaction 2-amino-4-hydroxypteridine + H2O ⇌ {\displaystyle \rightleftharpoons } 2,4-dihydroxypteridine + NH3 Thus, the two substrates of this enzyme are 2-amino-4-hydroxypteridine and H2O, whereas its two products are 2,4-dihydroxypteridine and NH3. (wikipedia.org)
Kinetic analysis of enzyme reactivation revealed an approximate doubling of enzyme Vmax with no change in enzyme affinity for either substrate, tryptophan, or pterin cofactor. (erowid.org)
Finally, the enzyme sepiapterin reductase (SR, PDB entry 1sep ) cleans up the portion of the sugar that is left to form the final cofactor. (rcsb.org)
Analysis of pterin nucleotides yielded a content of 1.3 +/- 0.1 guanine monophosphate/mol of enzyme, which suggests a tungsten coordination with two molybdopterin guanine dinucleotide cofactors. (unl.pt)
These biochemical reactions frequently involve cofactors, often vitamins, which help the specific enzyme function, e.g. vitamin B 6 is the cofactor for the enzyme cystathionine β synthase which converts the amino acid homocysteine into cystathionine. (oncohemakey.com)
BH4 is also a cofactor for tryptophan and serotonin synthesis, as well as for the enzyme nitrous oxide synthetase. (medscape.com)

Tetrahydrobiopterin (BH4) is a natural and essential cofactor for the enzymatic hydroxylation of phenylalanine (Phe) and tyrosine (Tyr), and for two tryptophan hydroxylases, three nitric oxide synthases, and glyceryl-ether monooxygenase. (thieme-connect.de)
Tetrahydrobiopterin (BH4) is an essential biological cofactor and a derivative of pterin which is considered potent anticancer agents. (bvsalud.org)
When used as a cofactor, tetrahydrobiopterin grabs molecular oxygen and attaches one oxygen atom to the target molecule, but it ends up having the other oxygen attached to itself in the process. (rcsb.org)
XDH is reported to have additional functions, i.E., The conversion of pterin to isoxanthopterin, one of the steps the degradation pathway of 5,6,7,8-tetrahydrobiopterin (BH4). (np-mrd.org)
Dopamine is produced from tyrosine by the action of tyrosine hydroxylase (TH) , which uses tetrahydrobiopterin (BH4) as a cofactor. (medscape.com)

The diagnosis is made by abnormal urinary pterin metabolites (low biopterin and high neopterin), reduced levels of cerebrospinal fluid (CSF) neurotransmitters homovanillic acid (HVA) and 5-hydroxy indoleacetic acid (5-HIAA) and the presence of a pathogenic mutation in the PTS gene. (rarediseasesjournal.com)

BH4 loading tests and measurement of neurotransmitter metabolites, pterins, and folates in cerebrospinal fluid can add further important information on disease severity. (thieme-connect.de)

After molybdopterin is eventually complexed with molybdenum, the complete ligand is usually called molybdenum cofactor. (wikipedia.org)
Molybdopterin consists of a pyranopterin, a complex heterocycle featuring a pyran fused to a pterin ring. (wikipedia.org)
Molybdenum cofactor biosynthesis occurs in four steps: (i) the radical-mediated cyclization of nucleotide, guanosine triphosphate (GTP), to (8S)‑3',8‐cyclo‑7,8‑dihydroguanosine 5'‑triphosphate (3',8‑cH2GTP), (ii) the formation of cyclic pyranopterin monophosphate (cPMP) from the 3',8‑cH2GTP, (iii) the conversion of cPMP into molybdopterin (MPT), (iv) the insertion of molybdate into MPT to form Moco. (wikipedia.org)
DMSO reductase, the metal is bound to two molybdopterin cofactors. (wikipedia.org)
Some bacterial oxidoreductases use tungsten in a similar manner as molybdenum by using it in a tungsten-pterin complex, with molybdopterin. (wikipedia.org)

Tetrahydrofolate (THF) and its one-carbon derivatives, collectively termed folates, are essential cofactors, but are inherently unstable. (ebi.ac.uk)
While it is clear that chemical oxidation can cleave folates or damage their pterin precursors, very little is known about enzymatic damage to these molecules or about whether the folate biosynthesis pathway responds adaptively to damage to its end-products. (ebi.ac.uk)
In silico modeling of the folate biosynthesis pathway showed that these observations are consistent with the in vivo cleavage of 5,10-methylene-THF by a side-activity of PanB, with FolK-mediated recycling of the pterin cleavage product, and with regulation of folate biosynthesis by folates or their damage products. (ebi.ac.uk)

An iron-molybdenum flavoprotein containing FLAVIN-ADENINE DINUCLEOTIDE that oxidizes hypoxanthine, some other purines and pterins, and aldehydes. (lookformedical.com)

The second, dihydropteridine reductase (DHPR, PDB entry 1dhr ), adds a few hydrogen atoms and the cofactor is ready to go for the next reaction. (rcsb.org)
1sep: G. Auerbach, A. Herrmann, M. Gutlich, M. Fischer, U. Jacob, A. Bacher & R. Huber (1997) The 1.25 A crystal structure of sepiapterin reductase reveals its binding mode to pterins and brain neurotransmitters. (rcsb.org)

In fact, the study of MPT models enables a deeper understanding of the "mechanism of function" of this cofactor and most importantly, lays the foundation for a potential treatment for the Moco related diseases MoCOD and iSOD. (uni-greifswald.de)

Genetic and metabolic analyses of Escherichia coli strains showed that overexpression of PanB leads to accumulation of the likely folate cleavage product 6-hydroxymethylpterin and other pterins in cells and medium, and-unexpectedly-to a 46% increase in total folate content. (ebi.ac.uk)

Finally, the Mtb DHPS structure reveals a highly conserved pterin binding pocket that may be exploited for the design of novel antimycobacterial agents. (dadospdf.com)

Molybdenum cofactor (Moco) biosynthesis is a highly conserved multistep pathway. (nih.gov)
Unlike many other cofactors, molybdenum cofactor (Moco) cannot be taken up as a nutrient. (wikipedia.org)
Molybdenum cofactor biosynthesis occurs in four steps: (i) the radical-mediated cyclization of nucleotide, guanosine triphosphate (GTP), to (8S)‑3',8‐cyclo‑7,8‑dihydroguanosine 5'‑triphosphate (3',8‑cH2GTP), (ii) the formation of cyclic pyranopterin monophosphate (cPMP) from the 3',8‑cH2GTP, (iii) the conversion of cPMP into molybdopterin (MPT), (iv) the insertion of molybdate into MPT to form Moco. (wikipedia.org)
The molybdenum cofactor (Moco) is essential for all kingdoms of life, plays central roles in various biological processes, and must be biosynthesized de novo. (duke.edu)
During Moco biosynthesis, the characteristic pyranopterin ring is constructed by a complex rearrangement of guanosine 5'-triphosphate (GTP) into cyclic pyranopterin (cPMP) through the action of two enzymes, MoaA and MoaC (molybdenum cofactor biosynthesis protein A and C, respectively). (duke.edu)
For catalytic activity, aldehyde oxidase requires a molybdo-pterin cofactor (molybdenum cofactor, MoCo) and flavin adenine dinucleotide. (cyprotex.com)
Eukaryotic molybdoenzymes use a unique molybdenum cofactor (MoCo) consisting of a pterin, termed molybdopterin, and the catalytically active metal molybdenum. (nih.gov)

The cofactor thus requires de novo biosynthesis. (wikipedia.org)
Scholars@Duke publication: Mechanism of pyranopterin ring formation in molybdenum cofactor biosynthesis. (duke.edu)
The encoded protein functions as both a dehydratase involved in tetrahydrobiopterin biosynthesis, and as a cofactor for HNF1A-dependent transcription. (nih.gov)
Tetrahydrofolate participates within a variety of biochem ical reactions and reduced folate cofactors are essential to the biosynthesis of a variety of molecules in each prokaryotes and eukaryotes. (cgrpreceptor.com)
magadii contained three genes en coding putative enzymes from the later on phases from the fol ate biosynthesis pathway. (cgrpreceptor.com)
While it is clear that chemical oxidation can cleave folates or damage their pterin precursors, very little is known about enzymatic damage to these molecules or about whether the folate biosynthesis pathway responds adaptively to damage to its end-products. (nih.gov)
In silico modeling of the folate biosynthesis pathway showed that these observations are consistent with the in vivo cleavage of 5,10-methylene-THF by a side-activity of PanB, with FolK-mediated recycling of the pterin cleavage product, and with regulation of folate biosynthesis by folates or their damage products. (nih.gov)
Sepiapterin reductase (SPR) catalyzes the reduction of sepiapterin to dihydrobiopterin (BH2), the precursor for tetrahydrobiopterin (BH4), a cofactor critical for nitric oxide biosynthesis and alkylglycerol and aromatic amino acid metabolism. (nih.gov)

Known structures of molybdo-pterin cofactor-containing enzymes will be described briefly and the structural differences between representatives of the same and different families will be analyzed. (studyres.com)
This comparison will show that the molybdo-pterin cofactor-containing enzymes represent a very heterogeneous group with differences in overall enzyme structure, cofactor composition and stoichiometry, as well as differences in the immediate molybdenum environment. (studyres.com)
Two recently discovered molybdo-pterin cofactor-containing enzymes will be described with regard to molecular and EPR spectroscopic properties, pyrogallol-phloroglucinol transhydroxylase from Pelobacter acidigallici and acetylene hydratase from Pelobacter acetylenicus. (studyres.com)

Molybdenum cofactor deficiency, a genetic illness. (wikipedia.org)
Molybdenum cofactor deficiency, which affects the functioning of sulfite oxidase, leads to a similar phenotype. (medscape.com)
However, many cases of this disorder are associated with deficiency of the molybdenum-containing pterin cofactor (molybdenum cofactor deficiency). (medscape.com)
Molybdenum cofactor deficiency. (medscape.com)
As sulfite oxidase deficiency and molybdenum cofactor deficiency have virtually identical phenotypes, the CNS toxicity appears to be secondary to loss of function of sulfite oxidase. (medscape.com)
Sulfite oxidase deficiency and molybdenum cofactor deficiency in the metabolism of sulfated amino acids. (medscape.com)
Closer to 100 cases of molybdenum cofactor deficiency have been reported. (medscape.com)
The estimated prevalence of molybdenum cofactor deficiency 1 per 100,000-200,000 newborns worldwide. (medscape.com)
Of note, some types of molybdenum cofactor deficiency have been responsive to treatment, leading to better longer-term outcomes. (medscape.com)
Isoxanthopterin is very low in some cases of hereditary xanthinuria (OMIM 278300 ) and molybdenum cofactor deficiency (OMIM 252150 ). (hmdb.ca)

Researchers believe that other enzymes may compensate for the reduced activity of pterin-4 alpha-carbinolamine dehydratase in people with PCBD1 gene mutations. (medlineplus.gov)
We used whole-genome sequencing with linkage analysis to study a consanguineous family with early-onset antibody-negative diabetes and identified a novel deletion in PCBD1 (pterin-4 α-carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 α), a gene that was recently proposed as a likely cause of diabetes. (diabetesjournals.org)

AbstractConjugated and unconjugated pteridines and their derivatives are important cofactors in cellular metabolism. (deepdyve.com)

Molybdenum cofactor is associated with the enzymes sulfite oxidase, xanthine dehydrogenase, and aldehyde oxidase (see the image below). (medscape.com)

These are polycyclic aromatic compounds containing a pterin moiety, which consist of a pteridine ring bearing a ketone and an amine group to form 2-aminopteridin-4(3H)-one. (hmdb.ca)

Catabolism of pteridine cofactors. (wikipedia.org)

The first step, the conversion of GTP to cyclic pyranopterin monophosphate (cPMP), requires the bicistronic gene molybdenum cofactor synthesis 1 ( MOCS1 ). (nih.gov)

As a result, less of this cofactor is available to participate in chemical reactions such as the conversion of phenylalanine to tyrosine. (medlineplus.gov)

Belongs to the class of organic compounds known as pterins and derivatives. (hmdb.ca)
Tetrahydrofolate (THF) and its one-carbon derivatives, collectively termed folates, are essential cofactors, but are inherently unstable. (nih.gov)

Molybdopterins are a class of cofactors found in most molybdenum-containing and all tungsten-containing enzymes. (wikipedia.org)

1. Introduction The molybdenum cofactor (Mo-co) is found in a variety of enzymes called hydroxylases or oxotransferases, most of which catalyze a net transfer of an oxygen atom to or from a substrate in a two electron transfer reaction [1^4]. (studyres.com)

AbstractThe analysis of pterins in the cerebrospinal fluid (CSF) is mandatory for the etiologic diagnosis of inborn errors of dopamine and serotonin metabolism. (deepdyve.com)

The manufacturing find out this here of folate includes numerous enzymes catalyzing the pterin and para aminobenzoic acid branches of your pathway. (cgrpreceptor.com)
Genetic and metabolic analyses of Escherichia coli strains showed that overexpression of PanB leads to accumulation of the likely folate cleavage product 6-hydroxymethylpterin and other pterins in cells and medium, and-unexpectedly-to a 46% increase in total folate content. (nih.gov)
Folic acid, also known as folate or Vitamin B9, is a member of the B vitamin family and an essential cofactor for enzymes involved in DNA and RNA synthesis. (rcsb.org)

Each B vitamin is either a cofactor (generally a coenzyme ) for key metabolic processes or is a precursor needed to make one. (wikipedia.org)

It requires the presence of the molybdenum cofactor for its proper functioning. (hmdb.ca)

Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2. (nih.gov)

Enzymes13

Dehydratase3

Deficiency10

Xanthine dehydrogenase1

Enzyme6

Tetrahydrobiopterin5

Biopterin1

Metabolites1

Molybdopterin5

Folates3

Dinucleotide1

Reductase2

Mechanism1

Metabolic1

Reveals1

Moco7

Biosynthesis8

Molybdo-pterin3

Molybdenum cofactor deficiency10

PCBD12

Pteridines1

Aldehyde oxidase1

Aromatic1

Pteridine1

MOCS11

Phenylalanine1

Derivatives2

Tungsten-contain1

Substrate1

Metabolism1

Folate3

Precursor1

Requires1

Formation1