Literature DB >> 6461312

Huntington's disease - imbalance of free amino acids in the cerebrospinal fluid of patients and offspring at-risk.

G Oepen, H Cramer, R Bernasconi, P Martin.   

Abstract

A total of 27 different amino acids were determined in the fasting, morning lumbar CSF of 12 patients with Huntington's Disease (HD), 8 at-risk offspring and 16 non-choreic control patients. A significant (P less than 0.001) decrease was observed for asparagine, isoleucine, leucine, phenylalanine, histidine, arginine, alpha-aminoadipic acid and homocarnosine in patients with HD compared to the non-choreic controls. Only tyrosine was increased in HD. These alterations were to an extent more pronounced in 5 neurophysiologically conspicuous offspring. The alterations suggest that amino acid imbalance is an early metabolic disturbance in HD.

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Year:  1982        PMID： 6461312     DOI： 10.1007/bf00343834

Source DB:  PubMed          Journal:  Arch Psychiatr Nervenkr (1970)


  17 in total

1.  Amino acid analysis of physiological fluids by a single-column programme based on stepwise elution with lithium citrate.

Authors:  R Tutschek; K D Meier; F Grüning; W Stubba
Journal:  J Chromatogr       Date:  1977-09-11

2.  Huntington's disease. Cerebrospinal fluid GABA levels in at-risk individuals.

Authors:  N V Manyam; T A Hare; L Katz; B S Glaeser
Journal:  Arch Neurol       Date:  1978-11

3.  CSF amino acids and plasma--CSF amino acid ratios in adults.

Authors:  T L Perry; S Hansen; J Kennedy
Journal:  J Neurochem       Date:  1975-03       Impact factor: 5.372

4.  Complicated optic atrophy (Bher's disease) associated with epilepsy and amino acid imbalance.

Authors:  F Monaco; A Pirisi; G P Sechi; R Mutani
Journal:  Eur Neurol       Date:  1979       Impact factor: 1.710

5.  The free amino acids in human cerebrospinal fluid.

Authors:  M van Sande; Y Mardens; K Adriaenssens; A Lowenthal
Journal:  J Neurochem       Date:  1970-02       Impact factor: 5.372

6.  Huntington's chorea-- measurements of somatostatin, substance P and cyclic nucleotides in the cerebrospinal fluid.

Authors:  H Cramer; J Kohler; G Oepen; G Schomburg; E Schröter
Journal:  J Neurol       Date:  1981       Impact factor: 4.849

7.  Cerebrospinal fluid homocarnosine in Huntington's disease.

Authors:  P Böhlen; G Tell; P J Schechter; J Koch-Weser; Y Agid; G Coquillat; G Chazot; C Fischer
Journal:  Life Sci       Date:  1980-03-31       Impact factor: 5.037

8.  Huntington's chorea. Post-mortem measurement of glutamic acid decarboxylase, choline acetyltransferase and dopamine in basal ganglia.

Authors:  E D Bird; L L Iversen
Journal:  Brain       Date:  1974-09       Impact factor: 13.501

9.  [Effect of administration of amino acids, especially of L-DOPA and -methyldopa, on the composition of cerebrospinal fluid in extrapyramidal syndromes. 3. Alterations of cerebrospinal fluid in patients with Huntington's chorea after -methyldopa or L-DOPA].

Authors:  M Simanyi; F Gerstenbrand; E Gründig; R Schedl; H Weiss
Journal:  Z Neurol       Date:  1973-03-16

10.  O-phthalaldehyde: fluorogenic detection of primary amines in the picomole range. Comparison with fluorescamine and ninhydrin.

Authors:  J R Benson; P E Hare
Journal:  Proc Natl Acad Sci U S A       Date:  1975-02       Impact factor: 11.205
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  3 in total

1.  Quantification of the Light Subunit of Neurofilament Protein in Cerebrospinal Fluid of Huntington's Disease Patients.

Authors:  Natalia Szejko; Carmen Picón; Juan García-Caldentey; Justo Garcia de Yebenes; Jose Carlos Alvarez-Cermeño; Luisa Maria Villar; José Luis López-Sendón Moreno
Journal:  PLoS Curr       Date:  2018-08-31

Review 2.  A Critical Evaluation of Wet Biomarkers for Huntington's Disease: Current Status and Ways Forward.

Authors:  Edina Silajdžić; Maria Björkqvist
Journal:  J Huntingtons Dis       Date:  2018

3.  Cross-sectional analysis of plasma and CSF metabolomic markers in Huntington's disease for participants of varying functional disability: a pilot study.

Authors:  Andrew McGarry; John Gaughan; Cory Hackmyer; Jacqueline Lovett; Mohammed Khadeer; Hamza Shaikh; Basant Pradhan; Thomas N Ferraro; Irving W Wainer; Ruin Moaddel
Journal:  Sci Rep       Date:  2020-11-24       Impact factor: 4.996
  3 in total

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