glycocyamine has been researched along with Amino Acid Metabolism Disorders, Inborn in 19 studies
glycocyamine: RN given refers to parent cpd; structure
guanidinoacetate : A monocarboxylic acid anion that is the conjugate base of guanidinoacetic acid, obtained by deprotonation of the carboxy group.
guanidinoacetic acid : The N-amidino derivative of glycine.
| Excerpt | Relevance | Reference |
|---|---|---|
| " Individuals with combined methylmalonic aciduria and homocystinuria have a functional impairment of the creatine synthetic pathway probably secondary to a relative depletion of labile methyl groups." | 7.73 | Creatine metabolism in combined methylmalonic aciduria and homocystinuria. ( Beaudet, AL; Bodamer, OA; Bottiglieri, T; O'Brien, WE; Sahoo, T; Scaglia, F; Stöckler-Ipsiroglu, S; Wagner, C, 2005) |
| " Individuals with combined methylmalonic aciduria and homocystinuria have a functional impairment of the creatine synthetic pathway probably secondary to a relative depletion of labile methyl groups." | 3.73 | Creatine metabolism in combined methylmalonic aciduria and homocystinuria. ( Beaudet, AL; Bodamer, OA; Bottiglieri, T; O'Brien, WE; Sahoo, T; Scaglia, F; Stöckler-Ipsiroglu, S; Wagner, C, 2005) |
| " AGAT patients might benefit from oral GAA due to upgraded bioavailability and convenient utilization of the compound, while possible drawbacks (e." | 2.61 | Benefits and drawbacks of guanidinoacetic acid as a possible treatment to replenish cerebral creatine in AGAT deficiency. ( Ostojic, SM, 2019) |
| "Treatment with creatine monohydrate (100-800 mg/kg/day) resulted in almost complete restoration of brain creatine levels and significant improvement of myopathy." | 1.42 | Arginine:glycine amidinotransferase (AGAT) deficiency: Clinical features and long term outcomes in 16 patients diagnosed worldwide. ( Apatean, D; Battini, R; DeBrosse, S; Dessoffy, K; Dowling, MD; Edvardson, S; Eichler, F; Johnston, K; Koeller, DM; Nouioua, S; Stockler-Ipsiroglu, S; Tazir, M; Verma, A; Wierenga, AM; Wierenga, KJ; Wong, LJ; Zhang, V, 2015) |
| "MR spectroscopy in a patient with argininosuccinate lyase deficiency revealed elevated cerebral guanidinoacetate signals, indicating that the phenomenon of increased levels of this compound in brain tissue is not limited to creatine deficiencies." | 1.33 | Cerebral 1H MR spectroscopy showing elevation of brain guanidinoacetate in argininosuccinate lyase deficiency. ( Oudkerk, M; Reijngoud, DJ; Sijens, PE; Soorani-Lunsing, RJ; van Spronsen, FJ, 2006) |
| "In two female siblings with mental retardation who had brain creatine deficiency that was reversible by means of oral creatine supplementation and had low urinary guanidinoacetate concentrations, AGAT deficiency was identified as a new genetic defect in creatine metabolism." | 1.31 | Arginine:glycine amidinotransferase deficiency: the third inborn error of creatine metabolism in humans. ( Alessandrì, MG; Bianchi, MC; Cioni, G; Fornai, F; Item, CB; Mühl, A; Stöckler-Ipsiroglu, S; Stromberger, C; Tosetti, M, 2001) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 1 (5.26) | 18.7374 |
| 1990's | 1 (5.26) | 18.2507 |
| 2000's | 9 (47.37) | 29.6817 |
| 2010's | 5 (26.32) | 24.3611 |
| 2020's | 3 (15.79) | 2.80 |
| Authors | Studies |
|---|---|
| Balestrino, M | 1 |
| Adriano, E | 1 |
| Kirby, T | 1 |
| Walters, DC | 1 |
| Brown, M | 1 |
| Jansen, E | 1 |
| Salomons, GS | 5 |
| Turgeon, C | 1 |
| Rinaldo, P | 1 |
| Arning, E | 1 |
| Ashcraft, P | 1 |
| Bottiglieri, T | 2 |
| Roullet, JB | 1 |
| Gibson, KM | 1 |
| Neu, A | 1 |
| Hornig, S | 1 |
| Sasani, A | 1 |
| Isbrandt, D | 1 |
| Gerloff, C | 1 |
| Tsikas, D | 1 |
| Schwedhelm, E | 1 |
| Choe, CU | 1 |
| Ostojic, SM | 1 |
| Nouioua, S | 2 |
| Cheillan, D | 2 |
| Zaouidi, S | 1 |
| Amedjout, N | 1 |
| Kessaci, F | 1 |
| Boulahdour, N | 1 |
| Hamadouche, T | 1 |
| Tazir, M | 2 |
| Joncquel-Chevalier Curt, M | 2 |
| Briand, G | 2 |
| Mention-Mulliez, K | 1 |
| Dobbelaere, D | 1 |
| Cuisset, JM | 1 |
| Lion-François, L | 1 |
| Des Portes, V | 1 |
| Chabli, A | 1 |
| Valayannopoulos, V | 1 |
| Benoist, JF | 1 |
| Pinard, JM | 1 |
| Simard, G | 1 |
| Douay, O | 1 |
| Deiva, K | 1 |
| Tardieu, M | 1 |
| Afenjar, A | 1 |
| Héron, D | 1 |
| Rivier, F | 1 |
| Chabrol, B | 1 |
| Prieur, F | 1 |
| Cartault, F | 1 |
| Pitelet, G | 1 |
| Goldenberg, A | 1 |
| Bekri, S | 1 |
| Gerard, M | 1 |
| Delorme, R | 1 |
| Porchet, N | 1 |
| Vianey-Saban, C | 1 |
| Vamecq, J | 1 |
| Stockler-Ipsiroglu, S | 3 |
| Apatean, D | 1 |
| Battini, R | 1 |
| DeBrosse, S | 1 |
| Dessoffy, K | 1 |
| Edvardson, S | 1 |
| Eichler, F | 1 |
| Johnston, K | 1 |
| Koeller, DM | 1 |
| Verma, A | 1 |
| Dowling, MD | 1 |
| Wierenga, KJ | 1 |
| Wierenga, AM | 1 |
| Zhang, V | 1 |
| Wong, LJ | 1 |
| Nasrallah, F | 1 |
| Kraoua, I | 1 |
| Bout, MA | 1 |
| Taieb, SH | 1 |
| Feki, M | 1 |
| Khouja, N | 1 |
| Kaabachi, N | 1 |
| Almeida, LS | 1 |
| Verhoeven, NM | 4 |
| Roos, B | 1 |
| Valongo, C | 1 |
| Cardoso, ML | 1 |
| Vilarinho, L | 1 |
| Jakobs, C | 4 |
| Arias, A | 1 |
| Garcia-Villoria, J | 1 |
| Ribes, A | 1 |
| Caldeira Araújo, H | 1 |
| Smit, W | 1 |
| Silva, S | 1 |
| Vasconcelos, R | 1 |
| Tomás, H | 1 |
| Tavares de Almeida, I | 1 |
| Duran, M | 1 |
| Bodamer, OA | 1 |
| Sahoo, T | 1 |
| Beaudet, AL | 1 |
| O'Brien, WE | 1 |
| Wagner, C | 1 |
| Scaglia, F | 1 |
| Sijens, PE | 2 |
| Reijngoud, DJ | 2 |
| Soorani-Lunsing, RJ | 2 |
| Oudkerk, M | 1 |
| van Spronsen, FJ | 2 |
| Braissant, O | 1 |
| Henry, H | 1 |
| Marescau, B | 1 |
| Lowenthal, A | 1 |
| Terheggen, HG | 1 |
| Esmans, E | 1 |
| Alderweireldt, F | 1 |
| Struys, EA | 1 |
| Jansen, EE | 1 |
| ten Brink, HJ | 1 |
| van der Knaap, MS | 1 |
| Item, CB | 1 |
| Stromberger, C | 1 |
| Mühl, A | 1 |
| Alessandrì, MG | 1 |
| Bianchi, MC | 1 |
| Tosetti, M | 1 |
| Fornai, F | 1 |
| Cioni, G | 1 |
| Schulze, A | 1 |
| Ebinger, F | 1 |
| Rating, D | 1 |
| Mayatepek, E | 1 |
2 reviews available for glycocyamine and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Benefits and drawbacks of guanidinoacetic acid as a possible treatment to replenish cerebral creatine in AGAT deficiency.
Topics: Amidinotransferases; Amino Acid Metabolism, Inborn Errors; Clinical Trials as Topic; Creatine; Devel | 2019 |
AGAT, GAMT and SLC6A8 distribution in the central nervous system, in relation to creatine deficiency syndromes: a review.
Topics: Amidinotransferases; Amino Acid Metabolism, Inborn Errors; Animals; Brain; Creatine; Developmental D | 2008 |
17 other studies available for glycocyamine and Amino Acid Metabolism Disorders, Inborn
| Article | Year |
|---|---|
Presence of guanidinoacetate may compensate creatine absence and account for less statin-induced muscle damage in GAMT-deficient compared to AGAT-deficient mice.
Topics: Amidinotransferases; Amino Acid Metabolism, Inborn Errors; Animals; Creatine; Developmental Disabili | 2020 |
Post-mortem tissue analyses in a patient with succinic semialdehyde dehydrogenase deficiency (SSADHD). I. Metabolomic outcomes.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Brain; Carnitine; Creatine; Creatinine; De | 2020 |
Creatine, guanidinoacetate and homoarginine in statin-induced myopathy.
Topics: Amidinotransferases; Amino Acid Metabolism, Inborn Errors; Animals; Creatine; Developmental Disabili | 2020 |
Creatine deficiency syndrome. A treatable myopathy due to arginine-glycine amidinotransferase (AGAT) deficiency.
Topics: Amidinotransferases; Amino Acid Metabolism, Inborn Errors; Brain; Child; Creatine; Developmental Dis | 2013 |
Creatine and guanidinoacetate reference values in a French population.
Topics: Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Amino Acid Metabolism, Inborn Errors; Case- | 2013 |
Arginine:glycine amidinotransferase (AGAT) deficiency: Clinical features and long term outcomes in 16 patients diagnosed worldwide.
Topics: Adolescent; Amidinotransferases; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Crea | 2015 |
Guanidinoacetate methyltransferase (GAMT) deficiency in two Tunisian siblings: clinical and biochemical features.
Topics: Abnormalities, Multiple; Adolescent; Amino Acid Metabolism, Inborn Errors; Child; Chromatography, Hi | 2012 |
Creatine and guanidinoacetate: diagnostic markers for inborn errors in creatine biosynthesis and transport.
Topics: Adolescent; Adult; Age Factors; Aged; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; | 2004 |
Guanidinoacetate and creatine/creatinine levels in controls and patients with urea cycle defects.
Topics: Age Factors; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Creatine; Gas Chromatogr | 2004 |
Guanidinoacetate methyltransferase deficiency identified in adults and a child with mental retardation.
Topics: Abnormalities, Multiple; Adult; Alleles; Amino Acid Metabolism, Inborn Errors; Child; Creatinine; DN | 2005 |
Creatine metabolism in combined methylmalonic aciduria and homocystinuria.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Creatine; Female; Glycine; Hom | 2005 |
Cerebral 1H MR spectroscopy showing elevation of brain guanidinoacetate in argininosuccinate lyase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Argininosuccinic Aciduria; Brain Chemistry; Child, Preschool; | 2006 |
High cerebral guanidinoacetate and variable creatine concentrations in argininosuccinate synthetase and lyase deficiency: implications for treatment?
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Argininosuccinate Synthase; Argininosuccinic Aciduri | 2006 |
Guanidino compounds in hyperargininemia.
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Chromatography, Liquid; Chromatography, Thin Layer; | 1982 |
An accurate stable isotope dilution gas chromatographic-mass spectrometric approach to the diagnosis of guanidinoacetate methyltransferase deficiency.
Topics: Amino Acid Metabolism, Inborn Errors; Child; Creatine; Gas Chromatography-Mass Spectrometry; Glycine | 1998 |
Arginine:glycine amidinotransferase deficiency: the third inborn error of creatine metabolism in humans.
Topics: Amidinotransferases; Amino Acid Metabolism, Inborn Errors; Amino Acid Sequence; Base Sequence; Brain | 2001 |
Improving treatment of guanidinoacetate methyltransferase deficiency: reduction of guanidinoacetic acid in body fluids by arginine restriction and ornithine supplementation.
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Child; Child, Preschool; Creatine; Dietary Supplemen | 2001 |