Article

CLN-1 and CLN-5, genes for infantile and variant late infantile neuronal ceroid lipofuscinoses, are expressed in the embryonic human brain

  1. Outi Heinonen1,2,*,
  2. Tarja Salonen1,
  3. Anu Jalanko1,
  4. Leena Peltonen1,3,
  5. Andrew Copp2

Article first published online: 11 SEP 2000

DOI: 10.1002/1096-9861(20001023)426:3<406::AID-CNE5>3.0.CO;2-5

Issue

The Journal of Comparative Neurology

The Journal of Comparative Neurology

Volume 426, Issue 3, pages 406–412, 23 October 2000

Additional Information

How to Cite

Heinonen, O., Salonen, T., Jalanko, A., Peltonen, L. and Copp, A. (2000), CLN-1 and CLN-5, genes for infantile and variant late infantile neuronal ceroid lipofuscinoses, are expressed in the embryonic human brain. The Journal of Comparative Neurology, 426: 406–412. doi: 10.1002/1096-9861(20001023)426:3<406::AID-CNE5>3.0.CO;2-5

Author Information

  1. 1

    National Public Health Institute, Department of Human Molecular Genetics, FIN-00300 Helsinki, Finland

  2. 2

    Neural Development Unit, Institute of Child Health, University College London, London WC1N 1EH, United Kingdom

  3. 3

    Department of Human Genetics, UCLA School of Medicine, Los Angeles, California 90024

*National Public Health Institute, Mannerheimintie 166, FIN-00300 Helsinki, Finland

Publication History

  1. Issue published online: 11 SEP 2000
  2. Article first published online: 11 SEP 2000
  3. Manuscript Accepted: 7 JUL 2000
  4. Manuscript Revised: 3 JUL 2000
  5. Manuscript Received: 21 OCT 1999

Funded by

  • Academy of Finland. Grant Number: 56210
  • Pediatric Research Foundation
  • Medical Research Council
  • the Wellcome Trust, U.K

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Keywords:

  • development;
  • immunohistochemistry;
  • in situ hybridization;
  • neurogenesis;
  • palmitoyl protein thioesterase

Abstract

Mutations in the CLN-1 and CLN-5 genes underlie the infantile, and Finnish variant of the late-infantile, neuronal ceroid lipofuscinoses, respectively. These disorders are characterized by a massive neuronal death early in childhood. We have studied mRNA and protein expression of CLN-1 and CLN-5 in embryonic human brains. The spatial and temporal distributions of CLN-1 and CLN-5 were similar in the embryonic human brain. Both genes are expressed at the beginning of cortical neurogenesis, and this expression increases as cortical development proceeds. In the developing cortical plate, expression is found in postmitotic migrating neuroblasts and neuroblasts that have completed migration. Expression was intense also in cells of the thalamus as well as in the future Purkinje cell layer of the cerebellum. These findings indicate that expression of CLN-1 and CLN-5 may be significant for development of a wide range of maturating neurons. J. Comp. Neurol. 426:406–412, 2000. © 2000 Wiley-Liss, Inc.

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