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Literature DB >> 3033396

Regional distribution of the phenylalanine-sensitive ATP-sulphurylase in brain.

Abstract

The regional distribution of the phenylalanine-sensitive ATP-sulphurylase in fetal calf brain coincides with demyelinated lesions observed in the central nervous systems of untreated PKU patients. This would be expected if this species of ATP-sulphurylase played a role in the pathogenesis of brain dysfunction in the untreated or poorly controlled phenylketonuria patient.

Entities: Disease Species

Mesh：

Substances：

Year: 1987 PMID： 3033396 DOI： 10.1007/BF01799489

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

16 in total

1. PHENYLKETONURIA PRESENTING AN INTERMITTENT PROGRESSIVE COURSE.

Authors: M BECHAR; B BORNSTEIN; M ELIAN; U SANDBANK
Journal: J Neurol Neurosurg Psychiatry Date: 1965-04 Impact factor: 10.154

2. Protein measurement with the Folin phenol reagent.

Authors: O H LOWRY; N J ROSEBROUGH; A L FARR; R J RANDALL
Journal: J Biol Chem Date: 1951-11 Impact factor: 5.157

3. Neuropathological findings in phenyl-pyruvic oligophrenia (phenyl-ketonuria).

Authors: E C ALVORD; L D STEVENSON; F S VOGEL; R L ENGLE
Journal: J Neuropathol Exp Neurol Date: 1950-07 Impact factor: 3.685

4. The assay of ATP-sulfurylase.

Authors: F A Hommes; L Moss
Journal: Anal Biochem Date: 1986-04 Impact factor: 3.365

5. p-Chlorophenylalanine treatment in developing rats: protein and lipids in whole brain and myelin.

Authors: I K Grundt; K Hole
Journal: Brain Res Date: 1974-07-12 Impact factor: 3.252

6. The protection of A1 myelin basic protein against the action of proteolytic enzymes after interaction of the protein with lipids at the air-water interface.

Authors: Y London; R A Demel; W S Geurts van Kessel; F G Vossenberg; L L van Deenen
Journal: Biochim Biophys Acta Date: 1973-07-18

Review 1. White matter abnormalities in patients with treated hyperphenylalaninaemia: magnetic resonance relaxometry and proton spectroscopy findings.

Authors: U Bick; K Ullrich; U Stöber; H Möller; G Schuierer; A C Ludolph; C Oberwittler; J Weglage; U Wendel
Journal: Eur J Pediatr Date: 1993-12 Impact factor: 3.183

1 in total

Regional distribution of the phenylalanine-sensitive ATP-sulphurylase in brain.

1. PHENYLKETONURIA PRESENTING AN INTERMITTENT PROGRESSIVE COURSE.

2. Protein measurement with the Folin phenol reagent.

3. Neuropathological findings in phenyl-pyruvic oligophrenia (phenyl-ketonuria).

4. The assay of ATP-sulfurylase.

5. p-Chlorophenylalanine treatment in developing rats: protein and lipids in whole brain and myelin.

6. The protection of A1 myelin basic protein against the action of proteolytic enzymes after interaction of the protein with lipids at the air-water interface.

7. Lipid composition of human cerebral white matter and myelin in phenylketonuria.

8. Brain protein and myelin metabolism in hyperphenylalaninemic rats.

9. Neuropathology of phenylketonuria.

10. Effect of experimental hyperphenylalaninemia on myelin metabolism at later stages of brain development.

Review 1. White matter abnormalities in patients with treated hyperphenylalaninaemia: magnetic resonance relaxometry and proton spectroscopy findings.