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Scholar Results 1 - 10 of about 101 related to AMBRUS: Extracorporeal enzyme reactors for depletion of phenylalanine in phenylketonuria. (0.09 sec) 

Extracorporeal enzyme reactors for depletion of phenylalanine in phenylketonuria


CM AMBRUS, S ANTHONE, C HORVATH, … - Annals of internal …, 1987 - Am Coll Physicians
Multitubular enzyme reactors with immobilized enzymes were developed to achieve depletion
of circulating substrate by extracorporeal means. To act as prototypes, reactors were prepared
with immobilized L-phenylalanine ammonia-lyase, an enzyme that metabolizes ...
Cited by 19 - Related articles - All 5 versions

Enzymatic control of phenylalanine intake in phenylketonuria.


JA Hoskins, G Jack, HE Wade, RJ Peiris, EC Wright, DJ … - Lancet, 1980 - ncbi.nlm.nih.gov
The plant enzyme phenylalanine ammonia lyase (PAL) will survive in the gut for long enough
to deplete the phenylalanine derived from food protein and so reduce the rise in blood phenylalanine
that otherwise occurs after a protein meal. This effect has been demonstrated in healthy ...
Cited by 31 - Related articles - All 3 versions

Phenylalanine ammonia-lyase immobilized in microcapsules for the depletion of …


L Bourget, TM Chang - Biochimica et biophysica acta, 1986 - ncbi.nlm.nih.gov
Microencapsulation of the enzyme phenylalanine ammonia-lyase was developed for in vivo
depletion of systemic phenylalanine in phenylketonuric rats. Compared to normal rats, systemic
phenylalanine blood levels in phenylketonuric rats was increased by 15-20-fold. Daily ...
Cited by 26 - Related articles - All 2 versions

The role of the blood-brain barrier in the aetiology of permanent brain dysfunction …


FA Hommes - Journal of Inherited Metabolic Disease, 1989 - Springer
Extensive investigations have provided evidence for the existence of two mechan- isms for the
transport of neutral amino acids (histidine, isoleucine, leucine, methion- ine, phenylalanine,
tryptophan, tyrosine and vatine) into the brain via the blood- brain barrier. The kinetic ...
Cited by 18 - Related articles - All 2 versions

[CITATION] Blood-brain barrier amino-acid transport: clinical implications


WM Pardridge - Inborn Errors of Metabolism in Humans: Monograph …, 1982 - AR Liss
Cited by 9 - Related articles - All 2 versions

Enzyme replacement therapy in ENU2 phenylketonuric mice using oral …


S Safos - Artificial Cells, Blood Substitutes and Biotechnology, 1995 - informahealthcare.com
The presence of an extensive enterorecirculation of amino acids between the intestine and the
body' allows the removal of systeniic phenylalanine in PKU rats by oral microencapsulated phenylalanine
ammonia lyase'. The work presented in this article has the main goal of assessing the ...
Cited by 18 - Related articles - BL Direct - All 4 versions

Effect of high-dose tyrosine supplementation on brain function in adults with …


J Pietz, R Landwehr, A Kutscha, H Schmidt, L de … - The Journal of pediatrics, 1995 - Elsevier
Nevertheless, in otherwise healthy patients with PKU, the motivation to adhere to a strict diet
usually decreases during adolescence despite medical recommendations. For patients who
are no longer able or willing to adhere to the diet, there has been a continuing search for “ ...
Cited by 27 - Related articles - BL Direct - All 9 versions

Nutritional value of essential amino acids in the treatment of adults with …


H Dotremont, B Francois, M Diels, P Gillis - Journal of inherited metabolic …, 1995 - Springer
Phenylketonuria (PKU; McKusick 261600) is an inherited metabolic disorder caused by a deficiency
of phenylalanine hydroxylase. Hyperphenylataninaemia results in progressive mental
deterioration, unless treatment with dietary phenylalanine (phe) restriction starts before 6 ...
Cited by 11 - Related articles - BL Direct - All 7 versions

A different approach to treatment of phenylketonuria: phenylalanine degradation …

- pnas.org
CN Sarkissian, Z Shao, F Blain, R … - Proceedings of the …, 1999 - National Acad Sciences
Phenylketonuria (PKU), with its associated hyperphenylalaninemia (HPA) and mental
retardation, is a classic genetic disease and the first to have an identified chemical cause of impaired
cognitive development. Treatment from birth with a low phenylalanine diet largely ...
Cited by 89 - Related articles - BL Direct - All 11 versions

[CITATION] First evidence that extra-cerebral recombinant phenylalanine ammonia lyase …


C Sarkissian, D Boulais, Z Shao, M Pedneault, R Heft, … - Am. J. Hum. Genet, 2003
Cited by 2 - Related articles


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