(Q34451232)

2 August 2017

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title

Phenylalanine ammonia lyase, enzyme substitution therapy for phenylketonuria, where are we now? (English)

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2 August 2017

1 reference

0 references

Christineh N Sarkissian

series ordinal

1

1 reference

PubMed ID

16165390

2 August 2017

Alejandra Gámez

series ordinal

2

1 reference

2 August 2017

13 September 2005

1 reference

Molecular Genetics and Metabolism

2 August 2017

1 reference

2 August 2017

86 Suppl 1

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2 August 2017

S22-6

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Measurement of phenyllactate, phenylacetate, and phenylpyruvate by negative ion chemical ionization-gas chromatography/mass spectrometry in brain of mouse genetic models of phenylketonuria and non-phenylketonuria hyperphenylalaninemia

2 August 2017

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

The influence of phenylalanine intake on the chemistry and behaviour of a phenyl-ketonuric child.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Treatment of phenylketonuria with a diet low in phenylalanine.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Studies on phenylketonuria. I. Restricted phenylalanine intake in phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

The use of gelatin capsules for ingestion of formula in dietary treatment of maternal phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Phenylketonuria due to phenylalanine hydroxylase deficiency: an unfolding story. Medical Research Council Working Party on Phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Recommendations on the dietary management of phenylketonuria. Report of Medical Research Council Working Party on Phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Recommendations for protein and amino acid intake in phenylketonuric patients

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Phenylketonuria: old disease, new approach to treatment

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Maternal phenylketonuria--results of dietary therapy.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Early breastfeeding is linked to higher intelligence quotient scores in dietary treated phenylketonuric children

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

The significance and mechanism of an increased serum phenylalanine-tyrosine ratio during infection

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

State-of-the-art 2003 on PKU gene therapy

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Correction of phenylketonuria after liver transplantation in a child with cirrhosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Brain phenylalanine concentration in the management of adults with phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Large neutral amino acid therapy and phenylketonuria: a promising approach to treatment

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Biopterin responsive phenylalanine hydroxylase deficiency

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

High frequency of tetrahydrobiopterin-responsiveness among hyperphenylalaninemias: a study of 1,919 patients observed from 1988 to 2002.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Tetrahydrobiopterin as an alternative treatment for mild phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

The metabolic and molecular bases of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Trends in enzyme therapy for phenylketonuria.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Toward PKU enzyme replacement therapy: PEGylation with activity retention for three forms of recombinant phenylalanine hydroxylase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Effect of pegylation on pharmaceuticals

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Complete nucleotide sequence of the Rhodosporidium toruloides gene coding for phenylalanine ammonia-lyase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Structural and catalytic properties of the four phenylalanine ammonia-lyase isoenzymes from parsley (Petroselinum crispum Nym.).

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Structure and characterization of a cDNA clone for phenylalanine ammonia-lyase from cut-injured roots of sweet potato

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

The phenylalanine ammonia-lyase gene family in Arabidopsis thaliana.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

The metabolism of cinnamic acid by healthy and phenylketonuric adults: a kinetic study

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Enzymatic control of phenylalanine intake in phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

The effect of proteinases on phenylalanine ammonia-lyase from the yeast Rhodotorula glutinis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Protection of phenylalanine ammonia-lyase from proteolytic attack

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Extracorporeal enzyme reactors for depletion of phenylalanine in phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Phenylalanine ammonia-lyase immobilized in microcapsules for the depletion of phenylalanine in plasma in phenylketonuric rat model

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Phenylalanine ammonia-lyase immobilized in semipermeable microcapsules for enzyme replacement in phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Therapeutic uses of microencapsulated genetically engineered cells.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Oral protein drug delivery

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Enzyme replacement therapy in ENU2 phenylketonuric mice using oral microencapsulated phenylalanine ammonia-lyase: a preliminary report

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Entrapment of phenylalanine ammonia-lyase in silk fibroin for protection from proteolytic attack.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Pahhph-5: a mouse mutant deficient in phenylalanine hydroxylase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

A heteroallelic mutant mouse model: A new orthologue for human hyperphenylalaninemia.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

A different approach to treatment of phenylketonuria: phenylalanine degradation with recombinant phenylalanine ammonia lyase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Crystal structure of the catalytic domain of human phenylalanine hydroxylase reveals the structural basis for phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Crystal structure and site-specific mutagenesis of pterin-bound human phenylalanine hydroxylase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Structural comparison of bacterial and human iron-dependent phenylalanine hydroxylases: similar fold, different stability and reaction rates

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Crystal structure of phenylalanine ammonia lyase: multiple helix dipoles implicated in catalysis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Development of pegylated forms of recombinant Rhodosporidium toruloides phenylalanine ammonia-lyase for the treatment of classical phenylketonuria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

A new amino acid mixture permits new approaches to the treatment of phenylketonuria.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

Regulation of phenylalanine ammonia lyase in Rhodotorula glutinis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2005.06.016

inferred from DOI database lookup

7 January 2021

based on heuristic

Identifiers

DOI

10.1016/J.YMGME.2005.06.016

1 reference

2 August 2017

1 reference

PubMed ID

16165390