scholarly journals Regulation of human cerebrospinal fluid malate dehydrogenase 1 in sporadic Creutzfeldt-Jakob disease patients

Aging ◽  
2016 ◽  
Vol 8 (11) ◽  
pp. 2927-2935 ◽  
Author(s):  
Matthias Schmitz ◽  
Franc Llorens ◽  
Alexander Pracht ◽  
Tobias Thom ◽  
Ângela Correia ◽  
...  
1973 ◽  
Vol 19 (2) ◽  
pp. 240-247 ◽  
Author(s):  
David M Sharpe ◽  
A Ross Wilcock ◽  
David M Goldberg

Abstract Optimum conditions with respect to pH and molarity of phosphate buffer, and the concentrations of substrates, coenzymes, and exogenous enzyme, were defined at 37°C for activity of aspartate transaminase and lactate dehydrogenase of human cerebrospinal fluid (CSF), as measured by optical kinetic assays at 340 nm. The resulting methods, and a previously published procedure for malate dehydrogenase activity applicable to human CSF, were adapted for use with an automatic reaction-rate monitor. Within-batch and between-batch precision of all methods was satisfactory, and repeated estimations in seven subjects showed good agreement. Freezing samples decreased their activity by 5 to 10%, but thereafter no further losses occurred at -20°C for as long as three months. Injection of up to 12 ml of air during encephalography had no major effect. Reference values, derived from subjects with no neurological or simple chronic degenerative CNS disease, suggested that the upper limits (in U/liter) are: aspartate transaminase, 13.5; lactate dehydrogenase, 40; malate dehydrogenase, 58.


Biomolecules ◽  
2019 ◽  
Vol 9 (12) ◽  
pp. 800 ◽  
Author(s):  
Inga Zerr ◽  
Anna Villar-Piqué ◽  
Vanda Edit Schmitz ◽  
Anna Poleggi ◽  
Maurizio Pocchiari ◽  
...  

The exploration of accurate diagnostic markers for differential diagnosis of neurodegenerative diseases is an ongoing topic. A previous study on cerebrospinal fluid (CSF)-mitochondrial malate dehydrogenase 1 (MDH1) in sporadic Creutzfeldt–Jakob disease (sCJD) patients revealed a highly significant upregulation of MDH1. Here, we measured the CSF levels of MDH1 via enzyme-linked immunosorbent assay in a cohort of rare genetic prion disease cases, such as genetic CJD (gCJD) cases, exhibiting the E200K, V210I, P102L (Gerstmann–Sträussler–Scheinker syndrome (GSS)), or D178N (fatal familial insomnia (FFI)) mutations in the PRNP. Interestingly, we observed enhanced levels of CSF-MDH1 in all genetic prion disease patients compared to neurological controls (without neurodegeneration). While E200K and V210I carriers showed highest levels of MDH1 with diagnostic discrimination from controls of 0.87 and 0.85 area under the curve (AUC), FFI and GSS patients exhibited only moderately higher CSF-MDH1 levels than controls. An impact of the PRNP codon 129 methionine/valine (MV) genotype on the amount of MDH1 could be excluded. A correlation study of MDH1 levels with other neurodegenerative marker proteins revealed a significant positive correlation between CSF-MDH1 concentration with total tau (tau) but not with 14-3-3 in E200K, as well as in V210I patients. In conclusion, our study indicated the potential use of MDH1 as marker for gCJD patients which may complement the current panel of diagnostic biomarkers.


1998 ◽  
Vol 39 (12) ◽  
pp. 2443-2451
Author(s):  
Kathleen S. Montine ◽  
Casey N. Bassett ◽  
Joyce J. Ou ◽  
William R. Markesbery ◽  
Larry L. Swift ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document