| SMART accession number: | SM00998
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| Description: |
Adenylosuccinate lyase catalyses two steps in the synthesis of purine nucleotides: the conversion of succinylaminoimidazole-carboxamide ribotide into aminoimidazole-carboxamide ribotide (the fifth step of de novo IMP biosynthesis); the formation of adenosine monophosphate (AMP) from adenylosuccinate (the final step in the synthesis of AMP from IMP). This entry represents the C-terminal, seven alpha-helical, domain of adenylosuccinate lyase. |
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| Interpro abstract (IPR019468): |
Adenylosuccinate lyase catalyses two steps in the synthesis of purine nucleotides: the conversion of succinylaminoimidazole-carboxamide ribotide into aminoimidazole-carboxamide ribotide (the fifth step of de novo IMP biosynthesis); the formation of adenosine monophosphate (AMP) from adenylosuccinate (the final step in the synthesis of AMP from IMP) [(PUBMED:17485188)]. This entry represents the seven alpha-helical, C-terminal domain of adenylosuccinate lyase [(PUBMED:9274883)]. It is also found in other enzymes, like in 3-carboxy-cis,cis-muconate cycloisomerase.
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| Family alignment: |
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Click on the following links for more information.
- Evolution (species in which this domain is found)
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- Cellular role (predicted cellular role)
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Cellular role: metabolism
- Literature (relevant references for this domain)
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Primary literature is listed below; Automatically-derived, secondary literature is also avaliable.
- Jurkiewicz E, Mierzewska H, Kusmierska K
- Adenylosuccinate lyase deficiency: the first identified polish patient.
- Brain Dev. 2007; 29: 600-2
- Display abstract
Adenylosuccinate lyase (ADSL) deficiency is a rare disease of de novopurine synthesis. The main symptoms are psychomotor retardation, epilepsy,autistic features, occasionally associated with muscular hypotonia.Diagnosis is made by detection of abnormal purine metabolites(succinyladenosine - S-Ado and succinylaminoimidazole carboxamide riboside- SAICAr) in body fluids. The severity of the clinical features correlateswith low S-Ado/SAICAr ratio. We report clinical, biochemical and brain MRIfindings of a female infant with severe early epilepsy and hypotonia, whodied at the age of 10 weeks.
- Marshall VM, Coppel RL
- Characterisation of the gene encoding adenylosuccinate lyase of Plasmodiumfalciparum.
- Mol Biochem Parasitol. 1997; 88: 237-41
- Structure (3D structures containing this domain)
3D Structures of ADSL_C domains in PDB
| PDB code | Main view | Title | | 1c3c |  | T. maritima adenylosuccinate lyase |
| 1c3u |  | T. maritima adenylosuccinate lyase |
| 1f1o |  | Structural studies of adenylosuccinate lyases |
| 1q5n |  | Crystal structure of beta-carboxy-cis,cis-muconate cycloisomerase (cmle) from acinetobacter calcoaceticus sp. adp1 |
| 1re5 |  | Crystal structure of 3-carboxy-cis,cis-muconate lactonizing enzyme from pseudomonas putida |
| 1yis |  | Structural genomics of caenorhabditis elegans: adenylosuccinate lyase |
| 2j91 |  | Crystal structure of human adenylosuccinate lyase in complex with amp |
| 2pfm |  | Crystal structure of adenylosuccinate lyase (purb) from bacillus anthracis |
| 2vd6 |  | Human adenylosuccinate lyase in complex with its substrate n6-(1,2-dicarboxyethyl)-amp, and its products amp and fumarate. |
| 3c8t |  | Crystal structure of fumarate lyase from mesorhizobium sp. bnc1 |
- Links (links to other resources describing this domain)
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to expand nodes. To display all proteins with a ADSL_C domain in a specific node, click on it.