(Q40865053)

24 September 2017

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title

Physiology and pathophysiology of organic acids in cerebrospinal fluid (English)

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24 September 2017

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Wolfram Meier-Augenstein

2

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24 September 2017

G F Hoffmann

1

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24 September 2017

S Stöckler

3

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24 September 2017

R Surtees

4

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24 September 2017

D Rating

5

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24 September 2017

W L Nyhan

6

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24 September 2017

1 January 1993

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Journal of Inherited Metabolic Disease

24 September 2017

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24 September 2017

16

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24 September 2017

648-669

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24 September 2017

4

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https://scigraph.springernature.com/pub.10.1007/bf00711898

24 September 2017

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cites work

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4-Hydroxybutyric aciduria in a patient without ataxia or convulsions

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Conservation of central nervous system glutaryl-coenzyme A dehydrogenase in fruit-eating bats with glutaric aciduria and deficient hepatic glutaryl-coenzyme A dehydrogenase

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O-(2,3,4,5,6-Pentafluorobenzyl)oxime-trimethylsilyl ester derivatives for sensitive identification and quantitation of aldehydes, ketones, and oxoacids in biological fluids

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Reference values for amino acids in cerebrospinal fluid of children determined using ion-exchange chromatography with fluorimetric detection

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The first adult case with 4-hydroxybutyric aciduria.

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Sudden infant death syndrome: organic acid profiles in cerebrospinal fluid from 47 children and the occurrence of N-acetylaspartic acid

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Therapeutic effect of sodium dichloroacetate on visual and auditory hallucinations in a patient with MELAS.

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Cerebrospinal fluid methylmalonic acid levels in normal subjects and patients with cobalamin deficiency

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12 December 2020

Kinetic analysis of the human blood-brain barrier transport of lactate and its influence by hypercapnia.

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Pipecolic acid pathway: The major lysine metabolic route in the rat brain

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L-2-hydroxyglutaric acidaemia: clinical and biochemical findings in 12 patients and preliminary report on L-2-hydroxyacid dehydrogenase.

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Carrier-mediated blood-brain barrier transport of short-chain monocarboxylic organic acids

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12 December 2020

Spectrum of total fatty acids in cerebrospinal fluid determined by gas chromatography

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12 December 2020

Significance of bound glutarate in the diagnosis of glutaric aciduria type I

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12 December 2020

Quantitative analysis for organic acids in biological samples: batch isolation followed by gas chromatographic-mass spectrometric analysis

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12 December 2020

Gammahydroxybutyrate: an endogenous regulator of energy metabolism

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12 December 2020

Organic acids in post-mortem cerebrospinal fluid

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12 December 2020

Quantitative analysis of polyols in human plasma and cerebrospinal fluid

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12 December 2020

O-(2,3,4,5,6-pentafluorobenzyl)oxime-trimethylsilyl ester derivatives for quantitative gas chromatographic and gas chromatographic-mass spectrometric studies of aldehydes, ketones and oxoacids

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12 December 2020

Organic acidurias: a review. Part 1.

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12 December 2020

Glutaric aciduria type I: Enzymatic and neuroradiologic investigations of two kindreds

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12 December 2020

Distribution of drugs between blood and cerebrospinal fluid: general methodology and effect of pH gradients.

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12 December 2020

Inhibition of brain glutamate decarboxylase by glutarate, glutaconate, and β-Hydroxyglutarate: Explanation of the symptoms in glutaric aciduria?

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12 December 2020

An inborn error of vitamin B12 metabolism associated with cellular deficiency of coenzyme forms of the vitamin. Pathological and neurochemical findings in one case.

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12 December 2020

Glutaric aciduria: biochemical and morphologic considerations.

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12 December 2020

"Cerebral" lactic acidosis: defects in pyruvate metabolism with profound brain damage and minimal systemic acidosis

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12 December 2020

Biotinidase deficiency: metabolites in CSF

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12 December 2020

CT and MR studies of methylmalonic acidemia

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12 December 2020

Medium‐chain triglycerides: a pitfall in the assay of organic acids in cerebrospinal fluid

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12 December 2020

Stable isotope dilution analysis of 4-hydroxybutyric acid: an accurate method for quantification in physiological fluids and the prenatal diagnosis of 4-hydroxybutyric aciduria

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12 December 2020

Direct analysis of tetrahydrobiopterin in cerebrospinal fluid by high-performance liquid chromatography with redox electrochemistry: prevention of autoxidation during storage and analysis

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12 December 2020

N-acetylaspartic aciduria: report of three new cases in children with a neurological syndrome associating macrocephaly and leukodystrophy.

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12 December 2020

Vigabatrin therapy in patient with succinic semialdehyde dehydrogenase deficiency.

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12 December 2020

Glutaric acidemia: a metabolic disorder causing progressive choreoathetosis

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12 December 2020

Permeability changes in the blood-brain barrier: causes and consequences

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12 December 2020

Changes during development in transport processes of the blood-brain barrier

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12 December 2020

Comparison of cytosolic and mitochondrial enzyme alterations in the livers of propionic or methylmalonic acidemia: a reduction of cytochrome oxidase activity

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12 December 2020

Adult-onset chorea and dementia with propionic acidemia

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12 December 2020

Phenotypic variability in glutaric aciduria type I: Report of fourteen cases in five Canadian Indian kindreds

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12 December 2020

Formation and absorption of cerebrospinal fluid in man

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12 December 2020

Glutaryl-coenzyme A dehydrogenase deficiency: a distinct encephalopathy

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12 December 2020

Identifiers

DOI

10.1007/BF00711898

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Dimensions Publication ID

24 September 2017

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