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2. The usefulness of pyridoxine in the treatment of homocystinuria: a review of postulated mechanisms of action and a new hypothesis. Spaeth GL Birth Defects Orig Artic Ser; 1976; 12(3):347-57. PubMed ID: 782596 [No Abstract] [Full Text] [Related]
3. Molecular basis of cystathionine beta-synthase deficiency in pyridoxine responsive and nonresponsive homocystinuria. Hu FL; Gu Z; Kozich V; Kraus JP; Ramesh V; Shih VE Hum Mol Genet; 1993 Nov; 2(11):1857-60. PubMed ID: 7506602 [TBL] [Abstract][Full Text] [Related]
4. Homocystinuria due to cystathionine beta-synthase deficiency--the effects of betaine treatment in pyridoxine-responsive patients. Wilcken DE; Dudman NP; Tyrrell PA Metabolism; 1985 Dec; 34(12):1115-21. PubMed ID: 3934499 [TBL] [Abstract][Full Text] [Related]
5. Functional modeling of vitamin responsiveness in yeast: a common pyridoxine-responsive cystathionine beta-synthase mutation in homocystinuria. Kim CE; Gallagher PM; Guttormsen AB; Refsum H; Ueland PM; Ose L; Folling I; Whitehead AS; Tsai MY; Kruger WD Hum Mol Genet; 1997 Dec; 6(13):2213-21. PubMed ID: 9361025 [TBL] [Abstract][Full Text] [Related]
6. Pyridoxine non-responsive p.R336C mutation alters the molecular properties of cystathionine beta-synthase leading to severe homocystinuria phenotype. Al-Sadeq DW; Thanassoulas A; Islam Z; Kolatkar P; Al-Dewik N; Safieh-Garabedian B; Nasrallah GK; Nomikos M Biochim Biophys Acta Gen Subj; 2022 Jul; 1866(7):130148. PubMed ID: 35417765 [No Abstract] [Full Text] [Related]
7. Studies on the use of skin fibroblasts for the measurement of cystathionine synthase activity with respect to homocystinuria. Griffiths R; Tudball N Clin Chim Acta; 1976 Nov; 73(1):157-62. PubMed ID: 1000825 [TBL] [Abstract][Full Text] [Related]
8. Free amino acids in liver of patients with homocystinuria due to cystathionine synthase deficiency: effects of vitamin B6. Rassin DK; Longhi RC; Gaull GE J Pediatr; 1977 Oct; 91(4):574-7. PubMed ID: 908976 [TBL] [Abstract][Full Text] [Related]
9. Recent advances in the mechanism of pyridoxine-responsive disorders. Fowler B J Inherit Metab Dis; 1985; 8 Suppl 1():76-83. PubMed ID: 3930845 [TBL] [Abstract][Full Text] [Related]
11. Homocystinuria--the effects of betaine in the treatment of patients not responsive to pyridoxine. Wilcken DE; Wilcken B; Dudman NP; Tyrrell PA N Engl J Med; 1983 Aug; 309(8):448-53. PubMed ID: 6877313 [TBL] [Abstract][Full Text] [Related]
12. CBS gene mutations found in a Chinese pyridoxine-responsive homocystinuria patient. Kwok JS; Fung SL; Lui GC; Law EL; Chan MH; Leung CB; Tang NL Pathology; 2011 Jan; 43(1):81-3. PubMed ID: 21240075 [No Abstract] [Full Text] [Related]
13. Electroencephalographic abnormalities in homocystinuria due to cystathionine synthase deficiency. Del Giudice E; Striano S; Andria G Clin Neurol Neurosurg; 1983; 85(3):165-8. PubMed ID: 6315290 [TBL] [Abstract][Full Text] [Related]
14. The molecular basis of cystathionine beta-synthase deficiency in Australian patients: genotype-phenotype correlations and response to treatment. Gaustadnes M; Wilcken B; Oliveriusova J; McGill J; Fletcher J; Kraus JP; Wilcken DE Hum Mutat; 2002 Aug; 20(2):117-26. PubMed ID: 12124992 [TBL] [Abstract][Full Text] [Related]
15. The intellectual abilities of early-treated individuals with pyridoxine-nonresponsive homocystinuria due to cystathionine beta-synthase deficiency. Yap S; Rushe H; Howard PM; Naughten ER J Inherit Metab Dis; 2001 Aug; 24(4):437-47. PubMed ID: 11596648 [TBL] [Abstract][Full Text] [Related]
16. Cystathionine beta-synthase deficiency: a qualitative abnormality of the deficient enzyme modified by vitamin B6 therapy. Longhi RC; Fleisher LD; Tallan HH; Gaull GE Pediatr Res; 1977 Feb; 11(2):100-3. PubMed ID: 840498 [TBL] [Abstract][Full Text] [Related]
17. A cautionary tale of pyridoxine toxicity in cystathionine beta-synthase deficiency detected by two-tier newborn screening highlights the need for clear pyridoxine dosing guidelines. Ames EG; Scott AJ; Pappas KB; Moloney SM; Conway RL; Ahmad A Am J Med Genet A; 2020 Nov; 182(11):2704-2708. PubMed ID: 32820583 [TBL] [Abstract][Full Text] [Related]
18. Strategies for the treatment of cystathionine beta-synthase deficiency: the experience of the Willink Biochemical Genetics Unit over the past 30 years. Walter JH; Wraith JE; White FJ; Bridge C; Till J Eur J Pediatr; 1998 Apr; 157 Suppl 2():S71-6. PubMed ID: 9587030 [TBL] [Abstract][Full Text] [Related]
19. Defective cystathionine beta-synthase regulation by S-adenosylmethionine in a partially pyridoxine responsive homocystinuria patient. Kluijtmans LA; Boers GH; Stevens EM; Renier WO; Kraus JP; Trijbels FJ; van den Heuvel LP; Blom HJ J Clin Invest; 1996 Jul; 98(2):285-9. PubMed ID: 8755636 [TBL] [Abstract][Full Text] [Related]
20. Cystathionine β-synthase deficiency in the E-HOD registry-part I: pyridoxine responsiveness as a determinant of biochemical and clinical phenotype at diagnosis. Kožich V; Sokolová J; Morris AAM; Pavlíková M; Gleich F; Kölker S; Krijt J; Dionisi-Vici C; Baumgartner MR; Blom HJ; Huemer M; J Inherit Metab Dis; 2021 May; 44(3):677-692. PubMed ID: 33295057 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]