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Selective Dysfunction of On- and/or Off-Bipolar Cell in Human Diseases

  • Yozo Miyake
  • Mineo Kondo
  • Makoto Nakamura
  • Hiroko Terasaki
Conference paper
  • 146 Downloads
Part of the Keio University International Symposia for Life Sciences and Medicine book series (KEIO, volume 11)

Abstract

In human, cone visual signals in the retina are processed through dual pathways, one involving ON-center bipolar cells (ON-pathway) and the other through OFF-center bipolar cells (the OFF-pathway), while rod visual signal is processed only through the ON-pathway. Little is known about how patients can see when the ON- and/or OFF-pathway is selectively defective. By comparing the monkey’s electroretinogram (ERG) of a blocked ON- or OFF-pathway using glutamate neurotransmitter analogs, we have detected new clinical entities in human where ON- and/or OFF-pathways are blocked in both rod and cone visual pathways. We hypothesized that the complete type of congenital stationary night blindness (CSNB 1) can be a model disease, having a complete dysfunction of only the ON-pathway, while the incomplete type (CSNB 2) has an incomplete dysfunction of both the ON- and OFF-pathways in both rod and cone visual systems. The gene mutation was detected in nyctalopin in CSNB 1, and in L-type calcium channel αl subunit in CSNB 2.

Key words

Bipolar cell dysfunction ERG Psychophysical function 

Copyright information

© Springer-Verlag Tokyo 2003

Authors and Affiliations

  • Yozo Miyake
    • 1
  • Mineo Kondo
    • 1
  • Makoto Nakamura
    • 1
  • Hiroko Terasaki
    • 1
  1. 1.Department of OphthalmologyNagoya University School of MedicineShowa-ku, NagoyaJapan

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