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Article

Correction of a mouse model of Menkes disease by the human Menkes gene.

Biochimica et biophysica acta - 1 Apr 2006

Llanos Roxana M, Ke Bi-Xia, Wright Magali, Deal Yolanda, Monty Francois, Kramer David R, Mercer Julian F B

Abstract excerpt

The brindled mouse is an accurate model of the fatal human X-linked copper deficiency disorder, Menkes disease. Males carrying the mutant allele of the Menkes gene orthologue Atp7a die in the second week of life. To determine whether the genetic defect in the brindled mice could be corrected by expression of the human Menkes gene, male transgenic mice expressing ATP7A from the chicken beta-actin composite...

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Identifiers

PMID: 16488577
DOI: 10.1016/j.bbadis.2005.12.011

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Correction of a mouse model of Menkes disease by the human Menkes gene.PMID 16488577Read ↗

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ATP7A gene addition to the choroid plexus results in long-term rescue of the lethal copper transport defect in a Menkes disease mouse model.Semantic proximity Read ↗

In vivo correction of a Menkes disease model using antisense oligonucleotides.Semantic proximity Read ↗

Autonomous requirements of the Menkes disease protein in the nervous system.Semantic proximity Read ↗

Maternofetal and neonatal copper requirements revealed by enterocyte-specific deletion of the Menkes disease proteinSemantic proximity Read ↗

Molecular and biochemical characterization of Mottled-dappled, an embryonic lethal Menkes disease mouse model.Semantic proximity Read ↗

Menkes disease: Oral administration of glyoxal-bis(N(4)-methylthiosemicarbazonato)-copper(II) rescues the macular mouse.Semantic proximity Read ↗

Prenatal treatment of mosaic mice (Atp7a mo-ms) mouse model for Menkes disease, with copper combined by dimethyldithiocarbamate (DMDTC).Semantic proximity Read ↗

Intravenous AAV9- ATP7A plus subcutaneous copper histidinate optimizes outcomes in a lethal Menkes disease mouse modelSemantic proximity Read ↗

Molecular genetics and pathophysiology of Menkes disease.Semantic proximity Read ↗

Conditional knockout of the Menkes disease copper transporter demonstrates its critical role in embryogenesis.Semantic proximity Read ↗

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