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Literature summary for 4.3.2.1 extracted from

  • Zheng, L.; Mackenzie, E.D.; Karim, S.A.; Hedley, A.; Blyth, K.; Kalna, G.; Watson, D.G.; Szlosarek, P.; Frezza, C.; Gottlieb, E.
    Reversed argininosuccinate lyase activity in fumarate hydratase-deficient cancer cells (2013), Cancer Metab., 1, 12.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 4.3.2.1

Application

Application Comment Organism
diagnostics the urea cycle metabolite argininosuccinate is a common metabolic biomarker of FH deficiency Mus musculus
diagnostics the urea cycle metabolite argininosuccinate is a common metabolic biomarker of FH deficiency Homo sapiens
medicine loss of function of fumarate hydratase, the mitochondrial tumor suppressor and tricarboxylic acid cycle enzyme, is associated with a highly malignant form of papillary and collecting duct renal cell cancer The accumulation of fumarate leads to reversed argininosuccinate lyase activity in fumarate hydratase-deficient cancer cells from Fh1-deficient humans, making these cells auxotrophic for arginine, which opens a therapeutic perspective for the cure of hereditary leiomyomatosis and renal cell cancer Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
fumarate + L-arginine Mus musculus accumulation of fumarate leads to reversed argininosuccinate lyase activity in fumarate hydratase-deficient cancer cells 2-(Nomega-L-arginino)succinate
-
 r
fumarate + L-arginine Homo sapiens accumulation of fumarate leads to reversed argininosuccinate lyase activity in fumarate hydratase-deficient cancer cells 2-(Nomega-L-arginino)succinate
-
 r

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
kidney
-
 Mus musculus
-
kidney
-
 Homo sapiens
-
kidney cancer cell
-
 Mus musculus
-
kidney cancer cell
-
 Homo sapiens
-
UOK-262 cell fumarate hydratase defiecient or proficient cells, the latter termed UOKpFH cells Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2-(Nomega-L-arginino)succinate
-
 Mus musculus fumarate + L-arginine
-
 r
2-(Nomega-L-arginino)succinate
-
 Homo sapiens fumarate + L-arginine
-
 r
fumarate + L-arginine accumulation of fumarate leads to reversed argininosuccinate lyase activity in fumarate hydratase-deficient cancer cells Mus musculus 2-(Nomega-L-arginino)succinate
-
 r
fumarate + L-arginine accumulation of fumarate leads to reversed argininosuccinate lyase activity in fumarate hydratase-deficient cancer cells Homo sapiens 2-(Nomega-L-arginino)succinate
-
 r

Synonyms

Synonyms Comment Organism
ASL
-
 Mus musculus
ASL
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction an increase in aspartate- and citrulline-derived argininosuccinate is a consequence of enzyme inhibition by high levels of fumarate in fumarate hydratase-deficient cells. In these cells, argininosuccinate is not produced from citrulline and aspartate in FH-deficient cells but rather from arginine and fumarate Mus musculus
malfunction argininosuccinate aciduria is observed in patients deficient in the enzyme. An increase in aspartate- and citrulline-derived argininosuccinate is a consequence of enzyme inhibition by high levels of fumarate in fumarate hydratase-deficient cells. In these cells, argininosuccinate is not produced from citrulline and aspartate in FH-deficient cells but rather from arginine and fumarate Homo sapiens
additional information depleting arginine from the growth media by the addition of pegylated arginine deiminase decreases the production of argininosuccinate in FH-deficient cells and reduces cell survival and proliferation Mus musculus
additional information depleting arginine from the growth media by the addition of pegylated arginine deiminase decreases the production of argininosuccinate in FH-deficient cells and reduces cell survival and proliferation Homo sapiens
physiological function loss of function of fumarate hydratase, the mitochondrial tumor suppressor and tricarboxylic acid cycle enzyme, is associated with a highly malignant form of papillary and collecting duct renal cell cancer. The accumulation of fumarate leads to reversed argininosuccinate lyase activity in fumarate hydratase-deficient cancer cells from Fh1-deficient humans, making these cells auxotrophic for arginine, which opens a therapeutic perspective for the cure of hereditary leiomyomatosis and renal cell cancer. The urea cycle metabolite argininosuccinate is a common metabolic biomarker of FH deficiency Homo sapiens
physiological function loss of function of fumarate hydratase, the mitochondrial tumor suppressor and tricarboxylic acid cycle enzyme, is associated with a highly malignant form of papillary and collecting duct renal cell cancer. The accumulation of fumarate leads to reversed argininosuccinate lyase activity in fumarate hydratase-deficient cancer cells from Fh1-deficient mice, making these cells auxotrophic for arginine Mus musculus