Chitotriosidase (CHIT1) is increased in microglia and macrophages in spinal cord of amyotrophic lateral sclerosis and cerebrospinal fluid levels correlate with disease severity and progression

@article{Steinacker2017ChitotriosidaseI,
  title={Chitotriosidase (CHIT1) is increased in microglia and macrophages in spinal cord of amyotrophic lateral sclerosis and cerebrospinal fluid levels correlate with disease severity and progression},
  author={Petra Steinacker and Federico Verde and Lubin Fang and Emily Feneberg and Patrick Oeckl and Sigrun Roeber and Sarah Anderl-Straub and Adrian Danek and Janine Diehl-Schmid and Klaus Fassbender and Klaus Fliessbach and Hans F{\"o}rstl and Armin Giese and Holger Jahn and Jan Kassubek and Johannes Kornhuber and Georg Bernhard Landwehrmeyer and Martin Lauer and Elmar H. Pinkhardt and Johannes Prudlo and Angela Rosenbohm and Anja Schneider and Matthias L. Schroeter and Hayrettin Tumani and Christine A. F. von Arnim and Jochen H Weishaupt and Patrick Weydt and Albert Christian Ludolph and Deniz Yilmazer Hanke and Markus Otto and Nibal Ackl and S{\"o}nke Arlt and Franziska Albrecht and Sandrine Bisenius and Svenja Gehlhaar and Jakob Gleiss and Theresa Halder and Jan T. Lehmbeck and Leonie Lampe and Johannes Levin and Manuel Maler and Felix Oberhauser and Timo J Oberstein and Catharina Prix and T. M. Raiser and Tanja Richter-Schmiedinger and Dorothee Saur and Lisa Schachner and Sonja Sch{\"o}necker and Katharina Schuemberg and Gisela Stenglein-Krapf and Elisabeth Wlasich},
  journal={Journal of Neurology, Neurosurgery, and Psychiatry},
  year={2017},
  volume={89},
  pages={239 - 247}
}
Objectives Neurochemical markers of amyotrophic lateral sclerosis (ALS) that reflect underlying disease mechanisms might help in diagnosis, staging and prediction of outcome. We aimed at determining the origin and differential diagnostic and prognostic potential of the putative marker of microglial activation chitotriosidase (CHIT1). Methods Altogether 316 patients were included, comprising patients with sporadic ALS, ALS mimics (disease controls (DCo)), frontotemporal lobar degeneration (FTLD… 
Chitotriosidase, a biomarker of amyotrophic lateral sclerosis, accentuates neurodegeneration in spinal motor neurons through neuroinflammation
TLDR
CHIT-1, an early diagnostic biomarker of sporadic ALS, activates glia priming them to attain a toxic phenotype resulting in neuroinflammation leading to motor neuronal death.
Different neuroinflammatory profile in amyotrophic lateral sclerosis and frontotemporal dementia is linked to the clinical phase
TLDR
The data indicate that neuroinflammation is linked to the symptomatic phase of ALS/FTD and shows a similar pattern in sporadic and genetic cases.
Cross-sectional and longitudinal measures of chitinase proteins in amyotrophic lateral sclerosis and expression of CHI3L1 in activated astrocytes
TLDR
CSF Chit-1 and CHI3L1 are significantly increased in ALS, and CSF ChIT-1and CHI 3L1 levels correlate to the rate of disease progression, and these cells were identified as a subset of activated astrocytes located predominately in the white matter of the motor cortex and the spinal cord.
Inflammatory markers in cerebrospinal fluid: independent prognostic biomarkers in amyotrophic lateral sclerosis?
TLDR
Findings show that inflammation in patients with ALS reflects the disease progression as an independent predictor of survival, and encourage the use of inflammatory markers in patient stratification and as surrogate markers of therapy response in clinical trials.
YKL40 in sporadic amyotrophic lateral sclerosis: cerebrospinal fluid levels as a prognosis marker of disease progression
TLDR
YKL40 and NF-L protein levels in the CSF are found to be a good biomarker combination of disease progression in sALS and Pearson’s correlation test linked positively the increased levels of YKL 40 with increasedNF-L levels.
Diagnostic-prognostic value and electrophysiological correlates of CSF biomarkers of neurodegeneration and neuroinflammation in amyotrophic lateral sclerosis
TLDR
Among all biomarkers, NfL yielded the highest diagnostic performance and was the best predictor of disease progression rate and survival in ALS and contribute to the understanding of the pathophysiological and electrophysiological correlates of biomarker changes.
Cerebrospinal Fluid YKL-40 and Chitotriosidase Levels in Frontotemporal Dementia Vary by Clinical, Genetic and Pathological Subtype
TLDR
Levels of CSF YKL-40 and chitotriosidase levels are increased in individuals with clinical FTD syndromes, particularly due to AD pathology, and in a preliminary analysis of genetic groups, levels of both proteins are found to be highly elevated in FTD due to GRN mutations, while YKl-40 is increased in Individuals with MAPT mutations.
Cerebrospinal Fluid Chitinases as Biomarkers for Amyotrophic Lateral Sclerosis
TLDR
The results supported the value of CHIT1 as a diagnostic and progression rate biomarker, and its potential as respiratory function marker, and opened novel perspectives to explore chitinases as biomarkers and their functional relevance in ALS.
CSF biomarkers of neuroinflammation in distinct forms and subtypes of neurodegenerative dementia
TLDR
CSF glial markers of neuroinflammation demonstrate limited diagnostic value but have some potential for monitoring the clinical and, possibly, preclinical phases of NDs.
Evaluation of Chitotriosidase and CC-Chemokine Ligand 18 as Biomarkers of Microglia Activation in Amyotrophic Lateral Sclerosis
TLDR
High ChT activity in CSF of patients with ALS may reflect microglia activation and could be a potential biomarker of the disease, and there was no correlation with the severity and progression of the Disease.
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