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Article

Canavan disease: studies on the knockout mouse.

Advances in experimental medicine and biology - 1 Jan 2006

Matalon Reuben, Michals-Matalon Kimberlee, Surendran Sankar, Tyring Stephen K

Abstract excerpt

Canavan disease (CD) is an autosomal recessive disorder, characterized by spongy degeneration of the brain. Patients with CD have aspartoacylase (ASPA) deficiency, which results accumulation of N-acetylaspartic acid (NAA) in the brain and elevated excretion of urinary NAA. Clinically, patients with CD have macrocephaly, mental retardation and hypotonia. A knockout mouse for CD which was engineered, also has ASPA...

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Source

Identifiers

PMID: 16802706
DOI: 10.1007/0-387-30172-0_6

Genes

ASPA

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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