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    
Abstract/OtherAbstract:
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.
Authors:
Graham W Kay; Marcel M Verbeek; Julie M Furlong; Michèl A A P Willemsen; David N Palmer
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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    
Affiliation:
Agriculture and Life Sciences Faculty, Lincoln University, Lincoln 7647, New Zealand.
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MeSH Terms
Descriptor/Qualifier:
Age Factors
Amino Acids / analysis,  cerebrospinal fluid*
Animals
Biological Markers / analysis,  cerebrospinal fluid
Child
Child, Preschool
Female
Glial Fibrillary Acidic Protein / analysis,  cerebrospinal fluid
Humans
Insulin-Like Growth Factor I / analysis,  cerebrospinal fluid
Male
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
Sheep
tau Proteins / analysis,  cerebrospinal fluid
Grant Support
ID/Acronym/Agency:
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
Chemical
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 4.2.1.11/Phosphopyruvate Hydratase
Comments/Corrections

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