taurine has been researched along with Homocystinuria in 13 studies
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)
| Excerpt | Relevance | Reference |
|---|---|---|
| "A phase 1/2 clinical trial was performed in individuals with cystathionine β synthase (CBS) deficient homocystinuria with aims to: (a) assess pharmacokinetics and safety of taurine therapy, (b) evaluate oxidative stress, inflammation, and vascular function in CBS deficiency, and (c) evaluate the impact of short-term taurine treatment." | 9.30 | Biomarkers of oxidative stress, inflammation, and vascular dysfunction in inherited cystathionine β-synthase deficient homocystinuria and the impact of taurine treatment in a phase 1/2 human clinical trial. ( Christians, U; Cowan, TM; Creadon-Swindell, G; Emmett, P; Ficicioglu, C; Freehauf, CL; Friederich, MW; Harrington, MJ; Henthorn, TK; Hite, M; Jiang, H; Kronquist, KE; MacLean, KN; Moreau, KL; Pena, LDM; Pyle, L; Spector, EB; Stabler, SP; Thomas, JA; Van Hove, JLK; Wempe, MF; Young, SP, 2019) |
| "Cystathionine β-synthase-deficient homocystinuria (HCU) is a serious life-threatening inborn error of sulfur metabolism with poorly understood pathogenic mechanisms." | 7.80 | Altered hepatic sulfur metabolism in cystathionine β-synthase-deficient homocystinuria: regulatory role of taurine on competing cysteine oxidation pathways. ( Abman, SH; Allen, RH; Jiang, H; Maclean, KN; Stabler, SP, 2014) |
| "Cystathionine was absent in the cerebrum of a patient with homocystinuria." | 7.64 | HOMOCYSTINURIA: ABSENCE OF CYSTATHIONINE IN THE BRAIN. ( GERRITSEN, T; WAISMAN, HA, 1964) |
| "Taurine treatment normalized the expression levels of γ-glutamyl ligase C/M, GS, OPLAH, and glyoxalase-1, and reversed HCU-induced deficits in protein glutathionylation by acting to double GSH levels relative to controls." | 5.48 | Taurine treatment prevents derangement of the hepatic γ-glutamyl cycle and methylglyoxal metabolism in a mouse model of classical homocystinuria: regulatory crosstalk between thiol and sulfinic acid metabolism. ( Aivazidis, S; Allen, RH; Harris, PS; Jiang, H; Kim, E; Maclean, KN; Petersen, DR; Roede, JR; Shearn, CT; Stabler, SP, 2018) |
| "A phase 1/2 clinical trial was performed in individuals with cystathionine β synthase (CBS) deficient homocystinuria with aims to: (a) assess pharmacokinetics and safety of taurine therapy, (b) evaluate oxidative stress, inflammation, and vascular function in CBS deficiency, and (c) evaluate the impact of short-term taurine treatment." | 5.30 | Biomarkers of oxidative stress, inflammation, and vascular dysfunction in inherited cystathionine β-synthase deficient homocystinuria and the impact of taurine treatment in a phase 1/2 human clinical trial. ( Christians, U; Cowan, TM; Creadon-Swindell, G; Emmett, P; Ficicioglu, C; Freehauf, CL; Friederich, MW; Harrington, MJ; Henthorn, TK; Hite, M; Jiang, H; Kronquist, KE; MacLean, KN; Moreau, KL; Pena, LDM; Pyle, L; Spector, EB; Stabler, SP; Thomas, JA; Van Hove, JLK; Wempe, MF; Young, SP, 2019) |
| "Cystathionine β-synthase-deficient homocystinuria (HCU) is a serious life-threatening inborn error of sulfur metabolism with poorly understood pathogenic mechanisms." | 3.80 | Altered hepatic sulfur metabolism in cystathionine β-synthase-deficient homocystinuria: regulatory role of taurine on competing cysteine oxidation pathways. ( Abman, SH; Allen, RH; Jiang, H; Maclean, KN; Stabler, SP, 2014) |
| "Cystathionine was absent in the cerebrum of a patient with homocystinuria." | 3.64 | HOMOCYSTINURIA: ABSENCE OF CYSTATHIONINE IN THE BRAIN. ( GERRITSEN, T; WAISMAN, HA, 1964) |
| "Taurine treatment normalized the expression levels of γ-glutamyl ligase C/M, GS, OPLAH, and glyoxalase-1, and reversed HCU-induced deficits in protein glutathionylation by acting to double GSH levels relative to controls." | 1.48 | Taurine treatment prevents derangement of the hepatic γ-glutamyl cycle and methylglyoxal metabolism in a mouse model of classical homocystinuria: regulatory crosstalk between thiol and sulfinic acid metabolism. ( Aivazidis, S; Allen, RH; Harris, PS; Jiang, H; Kim, E; Maclean, KN; Petersen, DR; Roede, JR; Shearn, CT; Stabler, SP, 2018) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 5 (38.46) | 18.7374 |
| 1990's | 2 (15.38) | 18.2507 |
| 2000's | 1 (7.69) | 29.6817 |
| 2010's | 5 (38.46) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Maclean, KN | 4 |
| Jiang, H | 4 |
| Aivazidis, S | 1 |
| Kim, E | 1 |
| Shearn, CT | 1 |
| Harris, PS | 1 |
| Petersen, DR | 1 |
| Allen, RH | 2 |
| Stabler, SP | 4 |
| Roede, JR | 1 |
| Phinney, WN | 1 |
| Keating, AK | 1 |
| Hurt, KJ | 1 |
| Van Hove, JLK | 1 |
| Freehauf, CL | 1 |
| Ficicioglu, C | 1 |
| Pena, LDM | 1 |
| Moreau, KL | 1 |
| Henthorn, TK | 1 |
| Christians, U | 1 |
| Cowan, TM | 1 |
| Young, SP | 1 |
| Hite, M | 1 |
| Friederich, MW | 1 |
| Spector, EB | 1 |
| Kronquist, KE | 1 |
| Thomas, JA | 1 |
| Emmett, P | 1 |
| Harrington, MJ | 1 |
| Pyle, L | 1 |
| Creadon-Swindell, G | 1 |
| Wempe, MF | 1 |
| Abman, SH | 1 |
| Kopecká, J | 1 |
| Krijt, J | 1 |
| Raková, K | 1 |
| Kožich, V | 1 |
| GERRITSEN, T | 1 |
| WAISMAN, HA | 1 |
| Mafrici, B | 1 |
| Reddy, GS | 1 |
| Wilcken, DE | 1 |
| Rödenbeck, D | 1 |
| Byrd, DJ | 1 |
| Brodehl, J | 1 |
| Jellum, E | 1 |
| Thorsrud, AK | 1 |
| Time, E | 1 |
| Hagberg, B | 1 |
| Hambraeus, L | 1 |
| Bensch, K | 1 |
| Heathcote, JG | 1 |
| Davies, DM | 1 |
| Haworth, C | 1 |
| Oliver, RW | 1 |
| Dunn, HG | 1 |
| Perry, TL | 1 |
| Dolman, CL | 1 |
1 trial available for taurine and Homocystinuria
| Article | Year |
|---|---|
Biomarkers of oxidative stress, inflammation, and vascular dysfunction in inherited cystathionine β-synthase deficient homocystinuria and the impact of taurine treatment in a phase 1/2 human clinical trial.
Topics: Adolescent; Adult; Biomarkers; Brachial Artery; Child; Cystathionine beta-Synthase; Female; Homocyst | 2019 |
12 other studies available for taurine and Homocystinuria
| Article | Year |
|---|---|
Taurine treatment prevents derangement of the hepatic γ-glutamyl cycle and methylglyoxal metabolism in a mouse model of classical homocystinuria: regulatory crosstalk between thiol and sulfinic acid metabolism.
Topics: Amino Acids; Aminobutyrates; Animals; Cystathionine beta-Synthase; Disease Models, Animal; Female; g | 2018 |
Taurine alleviates repression of betaine-homocysteine
Topics: Animals; Betaine; Betaine-Homocysteine S-Methyltransferase; Disease Models, Animal; Homocystinuria; | 2019 |
Altered hepatic sulfur metabolism in cystathionine β-synthase-deficient homocystinuria: regulatory role of taurine on competing cysteine oxidation pathways.
Topics: Animals; Blotting, Western; Carboxy-Lyases; Cystathionine beta-Synthase; Cysteine; Cysteine Dioxygen | 2014 |
Restoring assembly and activity of cystathionine β-synthase mutants by ligands and chemical chaperones.
Topics: Alleles; Aminolevulinic Acid; Betaine; Cystathionine beta-Synthase; Escherichia coli; Glycerol; Homo | 2011 |
HOMOCYSTINURIA: ABSENCE OF CYSTATHIONINE IN THE BRAIN.
Topics: Amino Acids; Brain; Child; Chromatography; Cystathionine; Cystathionine beta-Synthase; Cystine; Homo | 1964 |
The contribution of low plasma taurine to clinical complications of Homocystinurea.
Topics: Dietary Supplements; Homocystinuria; Humans; Models, Biological; Taurine | 2005 |
Experimental homocysteinemia in pigs: comparison with studies in sixteen homocystinuric patients.
Topics: Adolescent; Adult; Amino Acids; Aminobutyrates; Animals; Child; Child, Preschool; Dipeptides; Diseas | 1982 |
The plasma concentration and renal handling of taurine in healthy children and in pediatric patients with disturbed sulfur metabolism.
Topics: Adolescent; Child; Child, Preschool; Cystinosis; Cystinuria; Homocystinuria; Humans; Infant; Infant, | 1994 |
Capillary electrophoresis for diagnosis and studies of human disease, particularly metabolic disorders.
Topics: Biopsy; Chromatography, High Pressure Liquid; Cystinuria; Electrophoresis; Homocystinuria; Humans; M | 1991 |
A case of homocystinuria with a dystonic neurological syndrome.
Topics: Adolescent; Cystine; Extrapyramidal Tracts; Folic Acid; Homocystine; Homocystinuria; Humans; Male; M | 1970 |
An improved technique for the analysis of amino acids and related compounds on thin layers of cellulose. IV. The quantitative determination of amino acids in urine.
Topics: Amino Acid Metabolism, Inborn Errors; Autoanalysis; Cellulose; Child; Chromatography, Ion Exchange; | 1971 |
Homocystinuria. A recently discovered cause of mental defect and cerebrovascular thrombosis.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Animals; Blood; Blood Coagulation Disorders | 1966 |