Page last updated: 2024-11-07

penicillamine and Homocystinuria

penicillamine has been researched along with Homocystinuria in 7 studies

Penicillamine: 3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease.
penicillamine : An alpha-amino acid having the structure of valine substituted at the beta position with a sulfanyl group.

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
" In an attempt to remove bound homocyst(e)ine, D-penicillamine was given to three patients with pyridoxine-nonresponsive homocystinuria."3.66The effect of D-penicillamine on protein-bound homocyst(e)ine in homocystinurics. ( Curley, K; Kang, SS; Wong, PW, 1982)

Research

Studies (7)

TimeframeStudies, this research(%)All Research%
pre-19907 (100.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Kang, SS1
Wong, PW1
Curley, K1
Prockop, DJ1
Kivirikko, KI1
Tuderman, L1
Guzman, NA1
Rabenstein, DL1
Yamashita, GT1
PestaƱa, A1
Sandoval, IV1
Sols, A1
Eyre, DR1
Schaub, J1
Bremer, HJ1
Kelly, S1
Copeland, W1

Reviews

1 review available for penicillamine and Homocystinuria

ArticleYear
[Diet therapy of inborn errors of metabolism].
    Medizinische Klinik, 1970, Aug-01, Volume: 65, Issue:32

    Topics: Adult; Carbohydrate Metabolism, Inborn Errors; Child; Cystinosis; Diet Therapy; Dietary Carbohydrate

1970

Other Studies

6 other studies available for penicillamine and Homocystinuria

ArticleYear
The effect of D-penicillamine on protein-bound homocyst(e)ine in homocystinurics.
    Pediatric research, 1982, Volume: 16, Issue:5

    Topics: Adolescent; Adult; Blood Proteins; Female; Homocysteine; Homocystine; Homocystinuria; Humans; Male;

1982
The biosynthesis of collagen and its disorders (second of two parts).
    The New England journal of medicine, 1979, Jul-12, Volume: 301, Issue:2

    Topics: Aging; Animals; Colchicine; Collagen; Collagen Diseases; Ehlers-Danlos Syndrome; Glucosyltransferase

1979
Determination of homocysteine, penicillamine, and their symmetrical and mixed disulfides by liquid chromatography with electrochemical detection.
    Analytical biochemistry, 1989, Aug-01, Volume: 180, Issue:2

    Topics: Chromatography, High Pressure Liquid; Disulfides; Electrochemistry; Electrodes; Homocysteine; Homocy

1989
Inhibition by homocysteine of serine dehydratase and other pyridoxal 5'-phosphate enzymes of the rat through cofactor blockage.
    Archives of biochemistry and biophysics, 1971, Volume: 146, Issue:2

    Topics: Alanine Transaminase; Animals; Cysteine; Homocysteine; Homocystinuria; Humans; L-Serine Dehydratase;

1971
Collagen defects in genetic disorders of connective tissue.
    Developmental medicine and child neurology, 1974, Volume: 16, Issue:4

    Topics: Aminopropionitrile; Chemical Phenomena; Chemistry; Collagen; Ehlers-Danlos Syndrome; Homocystinuria;

1974
A hypothesis on the homocystinuric's response to pyridoxine.
    Metabolism: clinical and experimental, 1968, Volume: 17, Issue:9

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Chemistry, Clinical; Cysteine; Homocystinuria; Hum

1968