Aspartoacylase gene knockout results in severe vacuolation in the white matter and gray matter of the spinal cord in the mouse

Sankar Surendran, Gerald Campbell, Stephen K. Tyring, Reuben Matalon

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

Canavan disease (CD) is a neurodegenerative disorder characterized by the spongy degeneration of the white matter of the brain. Aspartoacylase (ASPA) gene mutation resulting enzyme deficiency is the basic cause of CD. Whether the ASPA defect in CD affects the spinal cord has been investigated using the ASPA gene knockout mouse. Luxol fast blue-hematoxylin and eosin staining in the spinal cord of the knockout mouse showed vacuolation in both white matter and gray matter areas of cervical, thoracic, lumbar, and sacral segments of the spinal cord. However, more vacuoles were seen in the gray matter than the white matter of the spinal cord. ASPA activity in the cervical, thoracic, lumbar, and sacrococcygeal regions of the spinal cord was significantly lower in the knockout mouse compared to the wild type. The enzyme defect in the knockout mouse was also confirmed using the Western blot method. These observations suggest that the ASPA gene defect in the mouse leads to spinal cord pathology, and that these changes may be partly involved in the cause of the physiological/behavioral abnormalities seen in the knockout mouse, if documented also in patients with CD.

Original languageEnglish (US)
Pages (from-to)385-389
Number of pages5
JournalNeurobiology of Disease
Volume18
Issue number2
DOIs
StatePublished - Mar 2005

Fingerprint

Gene Knockout Techniques
Canavan Disease
Spinal Cord
Knockout Mice
Thorax
Sacrococcygeal Region
Lumbosacral Region
Enzymes
Hematoxylin
Eosine Yellowish-(YS)
Vacuoles
Neurodegenerative Diseases
Genes
Gray Matter
White Matter
aspartoacylase
Western Blotting
Pathology
Staining and Labeling
Mutation

Keywords

  • Aspartoacylase
  • Canavan disease
  • Gray matter
  • Knockout mouse
  • Spinal cord vacuolation
  • Spongiform degeneration
  • White matter

ASJC Scopus subject areas

  • Neurology

Cite this

Aspartoacylase gene knockout results in severe vacuolation in the white matter and gray matter of the spinal cord in the mouse. / Surendran, Sankar; Campbell, Gerald; Tyring, Stephen K.; Matalon, Reuben.

In: Neurobiology of Disease, Vol. 18, No. 2, 03.2005, p. 385-389.

Research output: Contribution to journalArticle

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