Document Detail

Neuropeptide changes and neuroactive amino acids in CSF from humans and sheep with neuronal ceroid lipofuscinoses (NCLs, Batten disease).
MedLine Citation:
PMID:  19664668     Owner:  NLM     Status:  MEDLINE    
Anomalies in neuropeptides and neuroactive amino acids have been postulated to play a role in neurodegeneration in a variety of diseases including the inherited neuronal ceroid lipofuscinoses (NCLs, Batten disease). These are often indicated by concentration changes in cerebrospinal fluid (CSF). Here we compare CSF neuropeptide concentrations in patients with the classical juvenile CLN3 form of NCL and the classical late infantile CLN2 form with neuropeptide and neuroactive amino acid concentrations in CSF from sheep with the late infantile variant CLN6 form. A marked disease related increase in CSF concentrations of neuron specific enolase and tau protein was noted in the juvenile CLN3 patients but this was not observed in an advanced CLN2 patient nor CLN6 affected sheep. No changes were noted in S-100b, GFAP or MBP in patients or of S-100b, GFAP or IGF-1 in affected sheep. There were no disease related changes in CSF concentrations of the neuroactive amino acids, aspartate, glutamate, serine, glutamine, glycine, taurine and GABA in these sheep. The changes observed in the CLN3 patients may be progressive markers of neurodegeneration, or of underlying metabolic changes perhaps associated with CLN3 specific changes in neuroactive amino acids, as have been postulated. The lack of changes in the CLN2 and CLN6 subjects indicate that these changes are not shared by the CLN2 or CLN6 forms and changes in CSF concentrations of these compounds are unreliable as biomarkers of neurodegeneration in the NCLs in general.
Graham W Kay; Marcel M Verbeek; Julie M Furlong; Michèl A A P Willemsen; David N Palmer
Related Documents :
7595598 - Toxicity of sulphur-containing compounds to neuronal cell lines.
10082248 - Infantile spasm: the effect of corticotropin (acth) on the free amino acid profile in c...
3899278 - In vivo analysis of dopamine and its metabolites in the caudate nucleus during euthermi...
1931558 - 1h nmr characterization of normal human cerebrospinal fluid and the detection of methyl...
1784568 - Effects of feeding flax and two types of sunflower seeds on fatty acid compositions of ...
23107678 - Collagen characteristics of farmed atlantic salmon with firm and soft fillet texture.
Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2009-08-05
Journal Detail:
Title:  Neurochemistry international     Volume:  55     ISSN:  1872-9754     ISO Abbreviation:  Neurochem. Int.     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-10-20     Completed Date:  2010-01-25     Revised Date:  2013-06-02    
Medline Journal Info:
Nlm Unique ID:  8006959     Medline TA:  Neurochem Int     Country:  England    
Other Details:
Languages:  eng     Pagination:  783-8     Citation Subset:  IM    
Agriculture and Life Sciences Faculty, Lincoln University, Lincoln 7647, New Zealand.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Age Factors
Amino Acids / analysis,  cerebrospinal fluid*
Biological Markers / analysis,  cerebrospinal fluid
Child, Preschool
Glial Fibrillary Acidic Protein / analysis,  cerebrospinal fluid
Insulin-Like Growth Factor I / analysis,  cerebrospinal fluid
Myelin Basic Protein / analysis,  cerebrospinal fluid
Nerve Growth Factors / analysis,  cerebrospinal fluid
Neuronal Ceroid-Lipofuscinoses / cerebrospinal fluid*,  physiopathology
Neuropeptides / analysis,  cerebrospinal fluid*
Neurotransmitter Agents / analysis,  cerebrospinal fluid*
Phosphopyruvate Hydratase / analysis,  cerebrospinal fluid
Predictive Value of Tests
S100 Proteins / analysis,  cerebrospinal fluid
tau Proteins / analysis,  cerebrospinal fluid
Grant Support
NS053559,/NS/NINDS NIH HHS; R01 NS053559-01A1/NS/NINDS NIH HHS; R01 NS053559-02/NS/NINDS NIH HHS; R01 NS053559-03/NS/NINDS NIH HHS
Reg. No./Substance:
0/Amino Acids; 0/Biological Markers; 0/Glial Fibrillary Acidic Protein; 0/Myelin Basic Protein; 0/Nerve Growth Factors; 0/Neuropeptides; 0/Neurotransmitter Agents; 0/S-100 calcium-binding protein beta subunit; 0/S100 Proteins; 0/tau Proteins; 67763-96-6/Insulin-Like Growth Factor I; EC Hydratase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  NAP has no effect on spatial memory after short-term treatment in advanced stage Alzheimer's disease...
Next Document:  Immunosuppressive HLA-G molecule is upregulated in alveolar epithelial cells after influenza A virus...