Page last updated: 2024-10-19

racemethionine and Homocystinuria

racemethionine has been researched along with Homocystinuria in 3 studies

Racemethionine: A preparation of METHIONINE that includes a mixture of D-methionine and L-methionine isomers.

Homocystinuria: Autosomal recessive inborn error of methionine metabolism usually caused by a deficiency of CYSTATHIONINE BETA-SYNTHASE and associated with elevations of homocysteine in plasma and urine. Clinical features include a tall slender habitus, SCOLIOSIS, arachnodactyly, MUSCLE WEAKNESS, genu varus, thin blond hair, malar flush, lens dislocations, an increased incidence of MENTAL RETARDATION, and a tendency to develop fibrosis of arteries, frequently complicated by CEREBROVASCULAR ACCIDENTS and MYOCARDIAL INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, p979)

Research Excerpts

ExcerptRelevanceReference
"Classical homocystinuria (HCU) is a rare inborn error of amino acid metabolism characterized by accumulation of homocysteine, an intermediate product of methionine metabolism, leading to significant systemic toxicities, particularly within the vascular, skeletal, and ocular systems."8.31An orally administered enzyme therapeutic for homocystinuria that suppresses homocysteine by metabolizing methionine in the gastrointestinal tract. ( Aijaz, H; Du, F; Franklin, KA; Galanie, S; Guan, S; Hang, J; Huisman, G; Huitt-Roehl, C; Ismaili, MHA; Kruger, WD; Kruse, N; Lee, HO; McCluskie, K; Mitchell, V; Rajkovic, G; Silverman, AP; Skvorak, K; Teadt, L; Zhang, N, 2023)
"Classical homocystinuria (HCU) is an inborn defect of methionine metabolism caused by a deficiency of the enzyme cystathionine β-synthase (CBS)."4.31Relationship between Bone Mineral Density and Selected Parameters of Calcium-Phosphate Economy with Dietary Management and Metabolic Control in Polish Pediatric Patients with Classical Homocystinuria-A Preliminary Study. ( Batycka, M; Ehmke Vel Emczyńska-Seliga, E; Jaworski, M; Kobylińska, M; Lange, E; Lech, N; Lipiński, P; Perkowska, B; Pokora, P; Rokicki, D; Samborowska, E, 2023)
"Classical homocystinuria (HCU) is a rare inborn error of amino acid metabolism characterized by accumulation of homocysteine, an intermediate product of methionine metabolism, leading to significant systemic toxicities, particularly within the vascular, skeletal, and ocular systems."4.31An orally administered enzyme therapeutic for homocystinuria that suppresses homocysteine by metabolizing methionine in the gastrointestinal tract. ( Aijaz, H; Du, F; Franklin, KA; Galanie, S; Guan, S; Hang, J; Huisman, G; Huitt-Roehl, C; Ismaili, MHA; Kruger, WD; Kruse, N; Lee, HO; McCluskie, K; Mitchell, V; Rajkovic, G; Silverman, AP; Skvorak, K; Teadt, L; Zhang, N, 2023)

Research

Studies (3)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's3 (100.00)2.80

Authors

AuthorsStudies
Batycka, M1
Lange, E1
Ehmke Vel Emczyńska-Seliga, E1
Jaworski, M1
Kobylińska, M1
Lech, N1
Samborowska, E1
Lipiński, P1
Perkowska, B1
Pokora, P1
Rokicki, D1
Skvorak, K1
Mitchell, V1
Teadt, L1
Franklin, KA1
Lee, HO1
Kruse, N1
Huitt-Roehl, C1
Hang, J1
Du, F1
Galanie, S1
Guan, S1
Aijaz, H1
Zhang, N1
Rajkovic, G1
Kruger, WD1
Ismaili, MHA1
Huisman, G1
McCluskie, K1
Silverman, AP1
Uygur, E1
Aktuglu-Zeybek, C1
Aghalarov, M1
Cansever, MS1
Kıykım, E1
Zubarioglu, T1

Other Studies

3 other studies available for racemethionine and Homocystinuria

ArticleYear
Relationship between Bone Mineral Density and Selected Parameters of Calcium-Phosphate Economy with Dietary Management and Metabolic Control in Polish Pediatric Patients with Classical Homocystinuria-A Preliminary Study.
    Nutrients, 2023, Apr-27, Volume: 15, Issue:9

    Topics: Amino Acids; Bone Density; Calcium; Calcium Phosphates; Child; Cholecalciferol; Homocystinuria; Huma

2023
An orally administered enzyme therapeutic for homocystinuria that suppresses homocysteine by metabolizing methionine in the gastrointestinal tract.
    Molecular genetics and metabolism, 2023, Volume: 139, Issue:4

    Topics: Animals; Cystathionine beta-Synthase; Gastrointestinal Tract; Homocysteine; Homocystinuria; Humans;

2023
A Methionine-Portioning-Based Medical Nutrition Therapy with Relaxed Fruit and Vegetable Consumption in Patients with Pyridoxine-Nonresponsive Cystathionine-β-Synthase Deficiency.
    Nutrients, 2023, Jul-11, Volume: 15, Issue:14

    Topics: Cystathionine; Cystathionine beta-Synthase; Diet, Protein-Restricted; Fruit; Homocysteine; Homocysti

2023