Skip to content
MS
unreviewed

Cytosolic argininosuccinate synthetase (ASS1)

1 reactions · 1 pathways

Cofactors used

Clinical / pharmacological. Citrullinaemia type I

What it does, reaction by reaction

1 reactions

Urea Cycle Amino Acid & Nitrogen Metabolism · Mitochondrial matrix + cytosol

step 3 Irreversible

Citrulline + L-aspartate + ATP → argininosuccinate + AMP + PPᵢ

Converts Citrulline Aspartate ATP into Argininosuccinate AMP PPi

› Notes

Cytosolic argininosuccinate synthetase (ASS1) activates citrulline and adds aspartate. MgATP is cleaved to AMP + PPᵢ, making this an essentially irreversible step that consumes two additional high-energy phosphate equivalents.

Showing all 1 reactions.

What accelerates and inhibits it

Regulation is pathway-specific, so each context is listed separately

0 entries

No regulation recorded for this enzyme in the source documents.

Recent literature

Europe PMC · fetched just now · sorted by publication date

  1. 1
    Terpene synthases screening in agarwood tree <i>Aquilaria yunnanensis</i> leads to the discovery of agarospirol.

    Wen S, Cheng W, Li Y, Er L, Wu M, Deng Z, Lu L, Liu T. · 2026-06-17

    open access unreviewed
  2. 2
  3. 3
    Injectable microsphere-based delivery strategies for stem cells and their derivatives in tissue regeneration.

    Liang R, Chen J, Huang J, Fu P, Shi Z, Yue Y, Wang H, Lun H, Lin Q, Cao F, Zeng H, Zhang X, Lin J. · 2026-07-10

    open access unreviewed
  4. 4
    Soil-derived microbiota induces T regulatory cells and protect against mouse colitis, metabolic disease, and sepsis.

    Szurek EA, Ngo VL, Abo H, Cebula A, Chassaing B, Howard RA, Hart M, Hori S, Weaver CT, Gewirtz AT, Ignatowicz… · 2026-05-24

    cited 1× open access unreviewed
  5. 5
    Impact of leucine-rich diet on placenta's proteomic and metabolomic profiles of pregnant tumor-bearing rats.

    Dos Santos IF, Salgado CM, Carregari VC, Dos Santos RW, de Souza DM, Viana LR, Gomes-Marcondes MCC. · 2026-05-14

    open access unreviewed
  6. 6
    Arginine deprivation induces prolonged autophagy activation and ROS-dependent cell death in glioblastoma multiforme.

    Abdelkhalek M, El-Jawhari Y, Shaheen M, Saad R, Sarieddine M, El-Husseini N, El-Sibai M, Abi-Habib RJ. · 2026-05-07

    open access unreviewed
  7. 7
    The role of mesencephalic astrocyte-derived neurotrophic factor in digestive diseases.

    Pu S, Yan J, Wu T. · 2026-03-18

    open access unreviewed
  8. 8
    Dual Urea Cycle Dysfunction Caused by Coexisting Argininosuccinic Aciduria and Citrin Deficiency: Clinical, Biochemical, and Molecular Insights From an Exceptional Case.

    Zribi M, Bhouri M, Khatrouch S, Boudabous H, Häberle J, Abdelmoula MS, Chehida AB. · 2026-10-04

    open access unreviewed
  9. 9
    Essential Oral Single Nutritional Therapy Products for Inherited Metabolic Diseases: Evidence and Consensus Assessment Using a Modified Delphi Method.

    Stolwijk NN, Penninx BMF, Bosch AM, Langeveld M, Salomons GS, Hollak CEM, Essential Nutritional Therapy Produ… · 2026-11-01

    open access unreviewed
  10. 10

External claims. These come from an index outside this database and are not checked against it. Treat them as leads.