Shed CNTNAP2 ectodomain is detectable in CSF and regulates Ca2+ homeostasis and network synchrony via PMCA2/ATP2B2

Neuron. 2022 Feb 16;110(4):627-643.e9. doi: 10.1016/j.neuron.2021.11.025. Epub 2021 Dec 17.

Abstract

Although many neuronal membrane proteins undergo proteolytic cleavage, little is known about the biological significance of neuronal ectodomain shedding (ES). Here, we show that the neuronal sheddome is detectable in human cerebrospinal fluid (hCSF) and is enriched in neurodevelopmental disorder (NDD) risk factors. Among shed synaptic proteins is the ectodomain of CNTNAP2 (CNTNAP2-ecto), a prominent NDD risk factor. CNTNAP2 undergoes activity-dependent ES via MMP9 (matrix metalloprotease 9), and CNTNAP2-ecto levels are reduced in the hCSF of individuals with autism spectrum disorder. Using mass spectrometry, we identified the plasma membrane Ca2+ ATPase (PMCA) extrusion pumps as novel CNTNAP2-ecto binding partners. CNTNAP2-ecto enhances the activity of PMCA2 and regulates neuronal network dynamics in a PMCA2-dependent manner. Our data underscore the promise of sheddome analysis in discovering neurobiological mechanisms, provide insight into the biology of ES and its relationship with the CSF, and reveal a mechanism of regulation of Ca2+ homeostasis and neuronal network synchrony by a shed ectodomain.

Keywords: CNTNAP2; autism; bioinformatics; calcium; cerebrospinal fluid; ectodomain shedding; network dynamics; proteomics; schizophrenia; sheddome.

Publication types

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

MeSH terms

  • Autism Spectrum Disorder* / cerebrospinal fluid
  • Autism Spectrum Disorder* / genetics
  • Autism Spectrum Disorder* / metabolism
  • Cell Membrane / metabolism
  • Homeostasis
  • Humans
  • Membrane Proteins* / metabolism
  • Nerve Tissue Proteins* / metabolism
  • Neurons / metabolism
  • Plasma Membrane Calcium-Transporting ATPases* / cerebrospinal fluid
  • Plasma Membrane Calcium-Transporting ATPases* / genetics
  • Plasma Membrane Calcium-Transporting ATPases* / metabolism
  • Signal Transduction

Substances

  • CNTNAP2 protein, human
  • Membrane Proteins
  • Nerve Tissue Proteins
  • Plasma Membrane Calcium-Transporting ATPases
  • ATP2B2 protein, human