pyridoxal phosphate has been researched along with CBS Deficiency in 20 studies
Pyridoxal Phosphate: This is the active form of VITAMIN B 6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (PYRIDOXAMINE).
pyridoxal 5'-phosphate : The monophosphate ester obtained by condensation of phosphoric acid with the primary hydroxy group of pyridoxal.
| Excerpt | Relevance | Reference |
|---|---|---|
| "We investigated the effect of pyridoxine administration in three patients with homocystinuria due to cystathionine synthase deficiency." | 7.65 | Homocystinuria due to cystathionine synthase deficiency: the effect of pyridoxine. ( Brown, MS; Edwards, WA; Laster, L; Loeb, PM; Mudd, SH, 1970) |
| "The most common cause of severely elevated homocysteine or homocystinuria is inherited disorders in cystathionine beta-synthase." | 3.70 | Deletion of the regulatory domain in the pyridoxal phosphate-dependent heme protein cystathionine beta-synthase alleviates the defect observed in a catalytic site mutant. ( Banerjee, R; Kabil, O, 1999) |
| "We investigated the effect of pyridoxine administration in three patients with homocystinuria due to cystathionine synthase deficiency." | 3.65 | Homocystinuria due to cystathionine synthase deficiency: the effect of pyridoxine. ( Brown, MS; Edwards, WA; Laster, L; Loeb, PM; Mudd, SH, 1970) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 9 (45.00) | 18.7374 |
| 1990's | 5 (25.00) | 18.2507 |
| 2000's | 1 (5.00) | 29.6817 |
| 2010's | 4 (20.00) | 24.3611 |
| 2020's | 1 (5.00) | 2.80 |
| Authors | Studies |
|---|---|
| To-Figueras, J | 1 |
| Wijngaard, R | 1 |
| García-Villoria, J | 1 |
| Aarsand, AK | 1 |
| Aguilera, P | 1 |
| Deulofeu, R | 1 |
| Brunet, M | 1 |
| Gómez-Gómez, À | 1 |
| Pozo, OJ | 1 |
| Sandberg, S | 1 |
| Venincasa, VD | 1 |
| Sridhar, J | 1 |
| Krijt, J | 1 |
| Kopecká, J | 1 |
| Hnízda, A | 1 |
| Moat, S | 1 |
| Kluijtmans, LA | 1 |
| Mayne, P | 1 |
| Kožich, V | 2 |
| Smith, AT | 1 |
| Su, Y | 1 |
| Stevens, DJ | 1 |
| Majtan, T | 1 |
| Kraus, JP | 3 |
| Burstyn, JN | 1 |
| Yadav, PK | 1 |
| Xie, P | 1 |
| Banerjee, R | 2 |
| Chen, X | 1 |
| Wang, L | 1 |
| Fazlieva, R | 1 |
| Kruger, WD | 1 |
| Lipson, MH | 1 |
| Kraus, J | 2 |
| Rosenberg, LE | 2 |
| Applegarth, DA | 1 |
| Vallance, HD | 1 |
| Seccombe, D | 1 |
| Kabil, O | 1 |
| Fowler, B | 2 |
| Packman, S | 1 |
| Tanase, S | 1 |
| Morino, Y | 1 |
| Yoshida, I | 1 |
| Sakaguchi, Y | 1 |
| Nakano, M | 1 |
| Yamashita, F | 1 |
| Hitoshi, T | 1 |
| Pestaña, A | 1 |
| Sandoval, IV | 1 |
| Sols, A | 1 |
| Uhlendorf, BW | 1 |
| Conerly, EB | 1 |
| Mudd, SH | 2 |
| Frimpter, GW | 1 |
| Andelman, RJ | 1 |
| George, WF | 1 |
| Edwards, WA | 1 |
| Loeb, PM | 1 |
| Brown, MS | 1 |
| Laster, L | 1 |
| Yoshida, T | 1 |
| Tada, K | 1 |
| Yokoyama, Y | 1 |
| Arakawa, T | 1 |
3 reviews available for pyridoxal phosphate and CBS Deficiency
| Article | Year |
|---|---|
Biochemistry and molecular genetics of cystathionine beta-synthase deficiency.
Topics: Alleles; Animals; Cystathionine beta-Synthase; Homocystinuria; Humans; Molecular Biology; Mutation; | 1998 |
Recent advances in the mechanism of pyridoxine-responsive disorders.
Topics: Cells, Cultured; Cystathionine beta-Synthase; Fibroblasts; Homocystinuria; Humans; Hydro-Lyases; Pyr | 1985 |
Vitamin B6-dependency syndromes. New horizons in nutrition.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Aminobutyrates; Anemia, Sideroblastic; Animals; Child; | 1969 |
17 other studies available for pyridoxal phosphate and CBS Deficiency
| Article | Year |
|---|---|
Dysregulation of homocysteine homeostasis in acute intermittent porphyria patients receiving heme arginate or givosiran.
Topics: Acetylgalactosamine; Adult; Arginine; Cystathionine beta-Synthase; Female; Folic Acid; Heme; Homeost | 2021 |
Woman with sudden loss of vision: homocystinuria.
Topics: Adult; Blindness; Corneal Edema; Female; Homocysteine; Homocystinuria; Humans; Intraocular Pressure; | 2013 |
Determination of cystathionine beta-synthase activity in human plasma by LC-MS/MS: potential use in diagnosis of CBS deficiency.
Topics: Blood Chemical Analysis; Calibration; Case-Control Studies; Chromatography, Liquid; Cystathionine be | 2011 |
Effect of the disease-causing R266K mutation on the heme and PLP environments of human cystathionine β-synthase.
Topics: Catalytic Domain; Circular Dichroism; Cystathionine beta-Synthase; Electron Spin Resonance Spectrosc | 2012 |
Allosteric communication between the pyridoxal 5'-phosphate (PLP) and heme sites in the H2S generator human cystathionine β-synthase.
Topics: Allosteric Regulation; Binding Sites; Cystathionine beta-Synthase; Heme; Homocystinuria; Humans; Hyd | 2012 |
Contrasting behaviors of mutant cystathionine beta-synthase enzymes associated with pyridoxine response.
Topics: Alleles; Animals; Cystathionine beta-Synthase; Homocysteine; Homocystinuria; Humans; Kinetics; Mice; | 2006 |
Affinity of cystathionine beta-synthase for pyridoxal 5'-phosphate in cultured cells. A mechanism for pyridoxine-responsive homocystinuria.
Topics: Apoproteins; Cells, Cultured; Cystathionine beta-Synthase; Homocystinuria; Humans; Hydro-Lyases; Kin | 1980 |
Are patients with homocystinuria being missed?
Topics: Child; Chromatography, High Pressure Liquid; Diagnosis, Differential; Homocysteine; Homocystinuria; | 1995 |
Deletion of the regulatory domain in the pyridoxal phosphate-dependent heme protein cystathionine beta-synthase alleviates the defect observed in a catalytic site mutant.
Topics: Catalytic Domain; Cystathionine beta-Synthase; Hemeproteins; Homocysteine; Homocystinuria; Humans; M | 1999 |
Homocystinuria. Evidence for three distinct classes of cystathionine beta-synthase mutants in cultured fibroblasts.
Topics: Adolescent; Adult; Cell Line; Child; Child, Preschool; Cystathionine beta-Synthase; Female; Fibrobla | 1978 |
Pyridoxine responsive and unresponsive homocystinuria.
Topics: Base Sequence; Cells, Cultured; Cystathionine beta-Synthase; Genetic Testing; Homocystinuria; Humans | 1992 |
[Vitamin B6 dependency syndrome].
Topics: Amino Acid Metabolism, Inborn Errors; Aspartate Aminotransferases; Cystathionine; Homocystinuria; Hu | 1992 |
Pyridoxal phosphate-induced liver injury in a patient with homocystinuria.
Topics: Chemical and Drug Induced Liver Injury; Child; Homocystinuria; Humans; Male; Pyridoxal Phosphate | 1985 |
Inhibition by homocysteine of serine dehydratase and other pyridoxal 5'-phosphate enzymes of the rat through cofactor blockage.
Topics: Alanine Transaminase; Animals; Cysteine; Homocysteine; Homocystinuria; Humans; L-Serine Dehydratase; | 1971 |
Homocystinuria: studies in tissue culture.
Topics: Culture Techniques; Fibroblasts; Heterozygote; Homocysteine; Homocystine; Homocystinuria; Humans; Hy | 1973 |
Homocystinuria due to cystathionine synthase deficiency: the effect of pyridoxine.
Topics: Adult; Aldehydes; Autoanalysis; Carbon Isotopes; Chromatography; Cysteine; Diet; Diet Therapy; Enzym | 1970 |
Homocystinuria of vitamin B6 dependent type.
Topics: Amino Acids; Aspartate Aminotransferases; Child; Child, Preschool; Chromatography, Thin Layer; Elect | 1968 |