Mechanism of CAR syndrome: anti-recoverin antibodies are the inducers of retinal cell apoptotic death via the caspase 9- and caspase 3-dependent pathway

J Neuroimmunol. 2002 Nov;132(1-2):72-82. doi: 10.1016/s0165-5728(02)00314-4.

Abstract

Anti-recoverin autoantibodies have been associated with cancer-associated retinopathy (CAR), a paraneoplastic blinding disease. Those antibodies have been shown to induce apoptotic death of photoreceptor cells. The objective was to ascertain the mechanisms of retinal death induced by anti-recoverin antibody in vitro by examining the apoptotic pathway involved in retinal cell death. Internalization of anti-recoverin antibody or its Fab fragments by retinal cells mediated by endocytosis lead to cytotoxicity. Antibody cellular translocation induced the increase of bcl-x(s) and bax and the decrease in the bcl-x(L) protein. We detected the release of cytochrome c and down-regulation of the apaf-1 protein. This correlated with the sequential activation of caspase 9 and caspase 3, as well as the degradation of the caspase substrate PARP and the fragmentation of DNA. Our data show that anti-recoverin antibodies are inducers of apoptosis through the mitochondrial pathway involving caspases 9 and 3. We propose that a similar mechanism may be in place in patients with CAR syndrome where high levels of circulating antibodies have been associated with retinal degeneration.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Apoptosis
  • Apoptotic Protease-Activating Factor 1
  • Autoantibodies / immunology*
  • Calcium-Binding Proteins / immunology*
  • Caspase 3
  • Caspase 9
  • Caspases / physiology*
  • Cytochrome c Group / metabolism
  • DNA Fragmentation
  • Enzyme Activation
  • Eye Proteins*
  • Hippocalcin
  • Lipoproteins*
  • Nerve Tissue Proteins*
  • Paraneoplastic Syndromes / etiology*
  • Poly(ADP-ribose) Polymerases / metabolism
  • Proteins / analysis
  • Proto-Oncogene Proteins c-bcl-2 / biosynthesis
  • Rats
  • Recoverin
  • Retina / pathology*
  • Retinal Degeneration / etiology*

Substances

  • Apaf1 protein, rat
  • Apoptotic Protease-Activating Factor 1
  • Autoantibodies
  • Calcium-Binding Proteins
  • Cytochrome c Group
  • Eye Proteins
  • Lipoproteins
  • Nerve Tissue Proteins
  • Proteins
  • Proto-Oncogene Proteins c-bcl-2
  • Rcvrn protein, rat
  • Recoverin
  • Hippocalcin
  • Poly(ADP-ribose) Polymerases
  • Casp3 protein, rat
  • Casp9 protein, rat
  • Caspase 3
  • Caspase 9
  • Caspases