title: Increased Cerebrospinal Fluid Levels of Ubiquitin Carboxyl-Terminal Hydrolase L1 in Patients with Alzheimer's Disease.
abstract: BACKGROUND
  Dysfunctions of the ubiquitin proteasome system (UPS), including the highly abundant neuronal enzyme ubiquitin carboxyl-terminal hydrolase L1 (UCH-L1), and autophagy-related changes (lysosomal degradation) are implicated in several neurodegenerative disorders including Alzheimer's disease (AD).
  
  
  METHODS
  This study evaluated cerebrospinal fluid (CSF) levels of UCH-L1, protein deglycase (DJ-1), neuron-specific enolase (NSE), and tau phosphorylated at threonine 231 (P-tau231) in two independent patient and control cohorts. Cohort 1 included CSF samples from subjects having an AD biomarker profile (n = 10) or a control biomarker profile (n = 31), while cohort 2 was a monocenter clinical study including patients with AD (n = 32), mild cognitive impairment (n = 13), other dementias (n = 15), as well as cognitively healthy controls (n = 20).
  
  
  RESULTS
  UCH-L1 and P-tau231 were elevated in AD patients compared to controls in both cohorts. CSF levels of DJ-1 and NSE were unchanged in the AD group, whereas they were decreased in the group of other dementia compared to controls in the clinical study.
  
  
  CONCLUSIONS
  Our main findings support that the UPS pathway may be impaired in AD, and UCH-L1 may serve as an additional CSF biomarker for AD.
authors: Clinical Neurochemistry Laboratory, Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska University Hospital Mölndal, Sahlgrenska Academy, University of Gothenburg, Mölndal, Gothenburg, Sweden.
authors: Annika
authors: A
authors: Öhrfelt
authors: Department of Neuropsychiatry, Skaraborg Hospital, Falköping, Gothenburg, Sweden; Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
authors: Per
authors: P
authors: Johansson
authors: Clinical Neurochemistry Laboratory, Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska University Hospital Mölndal, Sahlgrenska Academy, University of Gothenburg, Mölndal, Gothenburg, Sweden.
authors: Anders
authors: A
authors: Wallin
authors: Clinical Neurochemistry Laboratory, Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska University Hospital Mölndal, Sahlgrenska Academy, University of Gothenburg, Mölndal, Gothenburg, Sweden.
authors: Ulf
authors: U
authors: Andreasson
authors: Clinical Neurochemistry Laboratory, Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska University Hospital Mölndal, Sahlgrenska Academy, University of Gothenburg, Mölndal, Gothenburg, Sweden; UCL Institute of Neurology, London, UK.
authors: Henrik
authors: H
authors: Zetterberg
authors: Clinical Neurochemistry Laboratory, Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska University Hospital Mölndal, Sahlgrenska Academy, University of Gothenburg, Mölndal, Gothenburg, Sweden.
authors: Kaj
authors: K
authors: Blennow
authors: Department of Endocrinology, Skaraborg Hospital, Skövde, Gothenburg, Sweden; Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
authors: Johan
authors: J
authors: Svensson
keywords: Alzheimer's disease; Biomarkers; Cerebrospinal fluid; DJ-1; Neuron-specific enolase; Tau phosphorylated at threonine 231; Ubiquitin carboxyl-terminal hydrolase L1