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Adenylosuccinate LyaseAdenylosuccinate SynthasePurine-Pyrimidine Metabolism, Inborn ErrorsLyasesAminoimidazole CarboxamidePurinesInosine MonophosphateRibonucleotidesAdenosine MonophosphateThermotoga maritimaBacillus subtilisMolecular Sequence DataKineticsMutation, MissenseAmino Acid Sequence

EnzymeAdenosineSynthetaseMutantsFumarateDeficiencySAICARProteinReactionStudiesAdenylosuccinaseSuccinyladenosine monophosphateAmino acidsGenePurineCell

Enzyme7

• Adenylosuccinate lyase (or adenylosuccinase) is an enzyme that in humans is encoded by the ADSL gene. (wikipedia.org)
• Adenylosuccinate lyase (ASL) is an enzyme that catalyzes two reactions in the de novo purine biosynthetic pathway. (wikipedia.org)
• The ADSL gene provides instructions for making an enzyme called adenylosuccinate lyase. (medlineplus.gov)
• Most of the mutations involved in this condition change single protein building blocks (amino acids) in the adenylosuccinate lyase enzyme. (medlineplus.gov)
• Zikanova M, Skopova V, Hnizda A, Krijt J, Kmoch S. Biochemical and structural analysis of 14 mutant adsl enzyme complexes and correlation to phenotypic heterogeneity of adenylosuccinate lyase deficiency. (medlineplus.gov)
• endothelin converting enzyme 2 [Source. (gsea-msigdb.org)
• A hydrolase enzyme that converts L-asparagine and water to L-aspartate and NH3. (lookformedical.com)

Adenosine4

• AICAR proceeds through three more reactions before it becomes adenylosuccinate (also called succinyladenosine monophosphate or SAMP), which ASL then splits into adenosine monophosphate (AMP) and fumarate. (wikipedia.org)
• Adenylosuccinate lyase in humans and Bacillus subtilis can be competitively inhibited by the substrate analog adenosine phosphonobutyric acid 2'(3'), 5'-diphosphate (APBADP). (wikipedia.org)
• In the ASL-catalyzed reaction splitting adenylosuccinate into adenosine monophosphate (AMP) and fumarate, the AMP must rotate slightly after the reaction is complete and before fumarate is released in order for both products to fit in the active site. (wikipedia.org)
• As part of this complex, adenylosuccinate lyase converts a molecule called succinylaminoimidazole carboxamide ribotide (SAICAR) to aminoimidazole carboxamide ribotide (AICAR) and converts succinyladenosine monophosphate (SAMP) to adenosine monophosphate (AMP). (medlineplus.gov)

Synthetase1

• It became apparent from early studies that 6-MP was catalyzed by hypoxanthine guanine phosphoribosyltransferase (HGPRTase) to 6-thioinosine 5'-phosphate(6-TIMP) which inhibited the enzymes, PRPP amidotransferase, IMP dehydrogenase, adenylosuccinate synthetase and adenylosuccinate lyase. (go.jp)

Mutants2

• Adenylosuccinate lyase mutants can have considerably reduced activity whether the mutation is in or away from the active site. (wikipedia.org)
• Biochemical and biophysical analysis of five disease-associated human adenylosuccinate lyase mutants. (medlineplus.gov)

Fumarate3

• Adenylosuccinate lyase converts adenylosuccinate to AMP and fumarate as part of the purine nucleotide cycle. (wikipedia.org)
• ASL cleaves adenylosuccinate into AMP and fumarate, and cleaves SAICAR into AICAR and fumarate. (wikipedia.org)
• In the first reaction, ASL converts 5-aminoimidazole- (N-succinylocarboxamide) ribotide (SAICAR) to 5-aminoimidazole-4-carboxamide ribotide (AICAR) and fumarate. (wikipedia.org)

Deficiency6

• Point mutations in adenylosuccinate that cause lowered enzymatic activity cause clinical symptoms that mark the condition adenylosuccinate lyase deficiency. (wikipedia.org)
• More than 50 mutations in the ADSL gene have been found to cause adenylosuccinate lyase deficiency. (medlineplus.gov)
• Detection of these substances in body fluids can help with diagnosis of adenylosuccinate lyase deficiency. (medlineplus.gov)
• damage to brain tissue caused by one or both of these substances likely underlies the neurological problems that occur in adenylosuccinate lyase deficiency. (medlineplus.gov)
• Studies suggest that the amount of SAICAr relative to S-Ado reflects the severity of adenylosuccinate lyase deficiency. (medlineplus.gov)
• Structural and biochemical characterization of human adenylosuccinate lyase (ADSL) and the R303C ADSL deficiency-associated mutation. (medlineplus.gov)

SAICAR2

• A reduction of adenylosuccinate lyase function, possibly due to a shortage of purinosomes, leads to buildup of SAICAR and SAMP. (medlineplus.gov)
• These substances are converted through a different reaction to succinylaminoimidazole carboxamide riboside (SAICAr) and succinyladenosine (S-Ado). (medlineplus.gov)

Protein1

• Adenylosuccinate lyase and other enzymes involved in purine synthesis form a group of proteins (a protein complex) called the purinosome. (medlineplus.gov)

Reaction1

• Adenylosuccinate lyase is part of the β-elimination superfamily of enzymes and it proceeds through an E1cb reaction mechanism. (wikipedia.org)

Studies1

• APBADP is a competitive inhibitor for both of the reactions catalyzed by adenylosuccinate lyase, and kinetic studies with APBADP show that the substrates for both reactions use the same active site. (wikipedia.org)

Adenylosuccinase1

• Adenylosuccinate lyase (or adenylosuccinase) is an enzyme that in humans is encoded by the ADSL gene. (wikipedia.org)

Succinyladenosine monophosphate2

• AICAR proceeds through three more reactions before it becomes adenylosuccinate (also called succinyladenosine monophosphate or SAMP), which ASL then splits into adenosine monophosphate (AMP) and fumarate. (wikipedia.org)
• As part of this complex, adenylosuccinate lyase converts a molecule called succinylaminoimidazole carboxamide ribotide (SAICAR) to aminoimidazole carboxamide ribotide (AICAR) and converts succinyladenosine monophosphate (SAMP) to adenosine monophosphate (AMP). (medlineplus.gov)

Amino acids2

• Most of the mutations involved in this condition change single protein building blocks (amino acids) in the adenylosuccinate lyase enzyme. (medlineplus.gov)
• TCA metabolism and FOCM enable cells to convert carbohydrates and amino acids to lipids, non-essential amino acids, nucleotides (including purines used for cofactor synthesis), glutathione, heme, and other cellular components. (biomedcentral.com)

Gene1

• The ADSL gene provides instructions for making an enzyme called adenylosuccinate lyase. (medlineplus.gov)

Purine2

• Adenylosuccinate lyase converts adenylosuccinate to AMP and fumarate as part of the purine nucleotide cycle. (wikipedia.org)
• Adenylosuccinate lyase and other enzymes involved in purine synthesis form a group of proteins (a protein complex) called the purinosome. (medlineplus.gov)

Cell1

• On the contrary, functional mitochondria are present in all proliferative cells within our body (including all tumors), as they are responsible for converting the diverse nutrients available to cells into the fundamental building blocks required for cell growth. (biomedcentral.com)