Class I and II histone deacetylase expression is not altered in human amyotrophic lateral sclerosis: Neuropathological and positron emission tomography molecular neuroimaging evidence

Muscle Nerve. 2019 Oct;60(4):443-452. doi: 10.1002/mus.26620. Epub 2019 Jul 16.

Abstract

Introduction: There is an unmet need for mechanism-based biomarkers and effective disease modifying treatments in amyotrophic lateral sclerosis (ALS). Previous findings have provided evidence that histone deacetylases (HDAC) are altered in ALS, providing a rationale for testing HDAC inhibitors as a therapeutic option.

Methods: We measured class I and II HDAC protein and transcript levels together with acetylation levels of downstream substrates by using Western blotting in postmortem tissue of ALS and controls. [11 C]Martinostat, a novel HDAC positron emission tomography ligand, was also used to assess in vivo brain HDAC alterations in patients with ALS and healthy controls (HC).

Results: There was no significant difference in HDAC levels between patients with ALS and controls as measured by Western blotting and reverse-transcription quantitative polymerase chain reaction. Similarly, no differences were detected in [11 C]Martinostat-positron emission tomography uptake in ALS participants compared with HCs.

Discussion: These findings provide evidence that alterations in HDAC isoforms are not a dominant pathological feature at the bulk tissue level in ALS.

Keywords: ALS; HDAC; PET imaging; acetylation; histone; postmortem.

Publication types

  • Observational Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adamantane / analogs & derivatives
  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / diagnostic imaging
  • Amyotrophic Lateral Sclerosis / genetics*
  • Amyotrophic Lateral Sclerosis / metabolism
  • Amyotrophic Lateral Sclerosis / pathology
  • Brain / diagnostic imaging
  • Brain / metabolism
  • Carbon Radioisotopes
  • Case-Control Studies
  • Cerebral Cortex / diagnostic imaging
  • Cerebral Cortex / metabolism
  • Cerebral Cortex / pathology
  • Cross-Sectional Studies
  • Female
  • Histone Deacetylases / genetics*
  • Histone Deacetylases / metabolism
  • Histones / metabolism
  • Humans
  • Hydroxamic Acids
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Molecular Imaging
  • Motor Cortex / diagnostic imaging
  • Motor Cortex / metabolism*
  • Motor Cortex / pathology
  • Multimodal Imaging
  • Positron-Emission Tomography
  • RNA, Messenger / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction
  • Spinal Cord / diagnostic imaging
  • Spinal Cord / metabolism*
  • Spinal Cord / pathology

Substances

  • Carbon Radioisotopes
  • Histones
  • Hydroxamic Acids
  • RNA, Messenger
  • martinostat
  • Histone Deacetylases
  • Adamantane