X-linked adrenoleukodystrophy: biochemical diagnosis and enzyme defect (Q35848817)

10 August 2017

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title

X-linked adrenoleukodystrophy: biochemical diagnosis and enzyme defect (English)

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X-linked adrenoleukodystrophy

10 August 2017

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van Roermund CW

2

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10 August 2017

R. J. Wanders

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1528022

5 October 2022

C. W. van Roermund

2

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1528022

5 October 2022

W. Lageweg

3

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5 October 2022

B. S. Jakobs

4

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5 October 2022

R. B. Schutgens

5

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5 October 2022

A. A. Nijenhuis

6

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5 October 2022

J. M. Tager

7

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5 October 2022

language of work or name

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1 January 1992

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Journal of Inherited Metabolic Disease

10 August 2017

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10 August 2017

15

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10 August 2017

4

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10 August 2017

634-644

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https://scigraph.springernature.com/pub.10.1007/bf01799620

10 August 2017

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cites work

Histochemical characteristics of the striated inclusions of adrenoleukodystrophy

1 reference

https://api.crossref.org/works/10.1007%2FBF01799620

Oxidation of very-long-chain fatty acids in rat brain: cerotic acid is beta-oxidized exclusively in rat brain peroxisomes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799620

Topography of very-long-chain-fatty-acid-activating activity in peroxisomes from rat liver

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799620

Adrenoleukodystrophy: increased plasma content of saturated very long chain fatty acids

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799620

Lignoceroyl-coenzyme A synthetase from developing rat brain: partial purification, characterization and comparison with palmitoyl-coenzyme A synthetase activity and liver enzyme.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799620

Adreno-leukodystrophy. Similar ultrastructural changes in adrenal cortical and Schwann cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799620

Adreno-leukodystrophy (sex-linked Schilder disease). Ultrastructural demonstration of specific cytoplasmic inclusions in the central nervous system

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799620

Acyl-CoA ligases from rat brain microsomes: an immunochemical study

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799620

Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders

21 January 2018

1 reference

12 December 2020

First prenatal diagnosis of acyl-CoA oxidase deficiency

1 reference

12 December 2020

Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids

1 reference

12 December 2020

Distinct long chain and very long chain fatty acyl CoA synthetases in rat liver peroxisomes and microsomes

1 reference

12 December 2020

Stable isotope dilution analysis of very long chain fatty acids in plasma, urine and amniotic fluid by electron capture negative ion mass fragmentography

1 reference

12 December 2020

Determination of very-long-chain fatty acids in plasma by a simplified gas chromatographic-mass spectrometric procedure

1 reference

12 December 2020

[ADDISON'S DISEASE WITH CEREBRAL SCLEROSIS IN CHILDHOOD. A HEREDITARY SYNDROME TRANSMITTED THROUGH CHROMOSOME X?]

1 reference

12 December 2020

Topographical localization of peroxisomal acyl-CoA ligases: differential localization of palmitoyl-CoA and lignoceroyl-CoA ligases

1 reference

12 December 2020

Conclusive evidence that very-long-chain fatty acids are oxidized exclusively in peroxisomes in human skin fibroblasts

1 reference

12 December 2020

Peroxisomal fatty acid oxidation in rat and human tissues. Effect of nutritional state, clofibrate treatment and postnatal development in the rat

1 reference

12 December 2020

Total and peroxisomal oxidation of various saturated and unsaturated fatty acids in rat liver, heart and m. quadriceps

1 reference

12 December 2020

Mitochondrial and peroxisomal beta-oxidation of stearic and lignoceric acids by rat brain

1 reference

12 December 2020

Direct transesterification of all classes of lipids in a one-step reaction

1 reference

12 December 2020

Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy

1 reference

12 December 2020

Increased Synthesis of Hexacosanoic Acid (C26 :0) by Cultured Skin Fibroblasts from Patients with Adrenoleukodystrophy (ALD) and Adrenomyeloneuropathy (AMN)1

1 reference

12 December 2020

Adrenoleukodystrophy: Elevated C26 fatty acid in cultured skin fibroblasts

1 reference

12 December 2020

Long-chain-acyl-CoA synthetase and very-long-chain-acyl-CoA synthetase activities in peroxisomes and microsomes from rat liver. An enzymological study

1 reference

12 December 2020

Immobilization of fatty acyl-CoA synthetase: effect on its stability and substrate specificity

1 reference

12 December 2020

Serum very long chain fatty acid pattern in Zellweger syndrome.

1 reference

12 December 2020

Very long chain fatty acid beta-oxidation by rat liver mitochondria and peroxisomes

1 reference

12 December 2020

Adrenoleukodystrophy: impaired oxidation of long chain fatty acids in cultured skin fibroblasts an adrenal cortex

1 reference

12 December 2020

A simple method for the isolation and purification of total lipides from animal tissues

1 reference

12 December 2020

Change of substrate specificity of rat liver microsomal fatty acyl-CoA synthetase activity by Triton X-100

1 reference

12 December 2020

Lignoceroyl-CoASH ligase: enzyme defect in fatty acid beta-oxidation system in X-linked childhood adrenoleukodystrophy

1 reference

12 December 2020

Very long chain fatty acid oxidation in rat liver

1 reference

12 December 2020

The inborn errors of peroxisomal beta-oxidation: a review

1 reference

12 December 2020

X-linked adrenoleukodystrophy: defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters

1 reference

12 December 2020

Fatty acid abnormality in adrenoleukodystrophy

1 reference

12 December 2020

A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy)

1 reference

12 December 2020

Fatty acid elongation activity in fibroblasts from patients with adrenoleukodystrophy (ALD)

1 reference

12 December 2020

Improved determination of very-long-chain fatty acids in plasma and cultured skin fibroblasts: applications to the diagnosis of peroxisomal disorders

1 reference

12 December 2020

Peroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy

1 reference

12 December 2020

Adrenoleukodystrophy. Preliminary report of a connatal case. Light- and electron microscopical, immunohistochemical and biochemical findings

1 reference

12 December 2020

Immunochemical and biochemical studies of fatty acid oxidation in fibroblasts of Zellweger and X-linked adrenoleukodystrophy patients

1 reference

12 December 2020

Purification and Properties of Long-Chain Acyl-Coenzyme-A Synthetase from Rat Liver

1 reference

12 December 2020

Peroxisomal disorders in neurology

1 reference

12 December 2020

Capillary gas-liquid chromatographic-mass spectrometric measurement of very long chain (C22 to C26) fatty acids in microliter samples of plasma

1 reference

12 December 2020

Identifiers

DOI

10.1007/BF01799620

1 reference

10 August 2017

1 reference