(Q43291024)

18 November 2017

title

Glyceryl triacetate for Canavan disease: a low-dose trial in infants and evaluation of a higher dose for toxicity in the tremor rat model (English)

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18 November 2017

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C N Madhavarao

1

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18 November 2017

P Arun

2

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18 November 2017

Y Anikster

3

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18 November 2017

S R Mog

4

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18 November 2017

O Staretz-Chacham

5

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18 November 2017

J R Moffett

6

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18 November 2017

N E Grunberg

7

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18 November 2017

W A Gahl

8

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18 November 2017

A M A Namboodiri

9

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18 November 2017

publication date

15 August 2009

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Journal of Inherited Metabolic Disease

18 November 2017

published in

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18 November 2017

volume

32

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18 November 2017

issue

5

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18 November 2017

page(s)

640-650

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Infantile CNS spongy degeneration--14 cases: clinical update

18 November 2017

cites work

1 reference

Crossref

https://api.crossref.org/works/10.1007%2FS10545-009-1155-3

Accumulation of N-acetyl-L-aspartate in the brain of the tremor rat, a mutant exhibiting absence-like seizure and spongiform degeneration in the central nervous system

21 January 2018

1 reference

12 December 2020

Immunohistochemical localization of aspartoacylase in the rat central nervous system

1 reference

12 December 2020

Time course of axonal myelination in the human brainstem auditory pathway

1 reference

12 December 2020

Natural history of Canavan disease revealed by proton magnetic resonance spectroscopy (1H-MRS) and diffusion-weighted MRI.

1 reference

12 December 2020

Progress toward acetate supplementation therapy for Canavan disease: glyceryl triacetate administration increases acetate, but not N-acetylaspartate, levels in brain

1 reference

12 December 2020

Magnetic resonance imaging and magnetic resonance spectroscopy in isolated sulfite oxidase deficiency

1 reference

12 December 2020

Esterase inhibition by grapefruit juice flavonoids leading to a new drug interaction

1 reference

12 December 2020

Characterization of the N-acetylaspartate biosynthetic enzyme from rat brain

1 reference

12 December 2020

Fats with short and medium chains. Physiological, biochemical, nutritional, and therapeutic aspects

1 reference

12 December 2020

Dietary intake of the short-chain triglyceride triacetin vs. long-chain triglycerides decreases adipocyte diameter and fat deposition in rats.

1 reference

12 December 2020

Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with canavan disease

1 reference

12 December 2020

N-acetylaspartic aciduria in a child with a progressive cerebral atrophy

1 reference

12 December 2020

Effect of parenteral administration of short-chain triglycerides on leucine metabolism

1 reference

12 December 2020

Brain N-acetylaspartate as a molecular water pump and its role in the etiology of Canavan disease: a mechanistic explanation

1 reference

12 December 2020

In vivo quantitation of cerebral metabolite concentrations using natural abundance 13C MRS at 1.5 T.

1 reference

12 December 2020

Final report on the safety assessment of triacetin

1 reference

12 December 2020

Identification and distribution of aspartoacylase in the postnatal rat brain

1 reference

12 December 2020

Rapid and sensitive screening for and chemical diagnosis of Canavan disease by gas chromatography-mass spectrometry

1 reference

12 December 2020

Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease.

1 reference

12 December 2020

Quantitative measurements with localized 1H MR spectroscopy in children with Canavan's disease

1 reference

12 December 2020

Mutational analysis of aspartoacylase: implications for Canavan disease

1 reference

12 December 2020

Intraneuronal N-acetylaspartate supplies acetyl groups for myelin lipid synthesis: evidence for myelin-associated aspartoacylase

1 reference

12 December 2020

Expression of N-acetylaspartate and N-acetylaspartylglutamate in the nervous system

1 reference

12 December 2020

Canavan disease: genomic organization and localization of human ASPA to 17p13-ter and conservation of the ASPA gene during evolution

1 reference

12 December 2020

Knock-out mouse for Canavan disease: a model for gene transfer to the central nervous system

1 reference

12 December 2020

Preimplantation genetic diagnosis of Canavan disease.

1 reference

12 December 2020

Myelin lipid abnormalities in the aspartoacylase-deficient tremor rat.

1 reference

12 December 2020

Acetate metabolism in the nervous system. N-acetyl-L-aspartic acid and the biosynthesis of brain lipids.

1 reference

12 December 2020

A case of Canavan disease: the first biochemically proven case in a Japanese girl

1 reference

12 December 2020

N-acetylaspartic aciduria due to aspartoacylase deficiency--a new aetiology of childhood leukodystrophy

1 reference

12 December 2020

N-Acetyl-L-aspartic acid in brain.

1 reference

12 December 2020

N-Acetyl-<i>L</i>-Aspartate is a Major Source of Acetyl Groups for Lipid Synthesis during Rat Brain Development

1 reference

12 December 2020

N-acetylaspartate as an acetyl source in the nervous system

1 reference

12 December 2020

Differential distribution of N-acetylaspartylglutamate and N-acetylaspartate immunoreactivities in rat forebrain

1 reference

12 December 2020

Acetyl transport mechanisms. Involvement of N-Acetyl aspartic acid in de novo fatty acid biosynthesis in the developing rat brain

1 reference

12 December 2020

N-Acetylaspartate in the CNS: from neurodiagnostics to neurobiology

1 reference

12 December 2020

10.1007/S10545-009-1155-3

Identifiers

DOI

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Dimensions Publication ID

18 November 2017

1046620640

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