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Article

A transgenic mouse model for human hereditary neuropathy with liability to pressure palsies.

Molecular and cellular neurosciences - 1 Jan 1997

Maycox P R, Ortuño D, Burrola P, Kuhn R, Bieri P L, Arrezo J C, Lemke G

Abstract excerpt

Mutations in the gene encoding peripheral myelin protein 22 (PMP22) account for several inherited peripheral neuropathies in humans. We now show that transgenic mice expressing antisense PMP22 RNA exhibit modestly reduced levels of PMP22 together with a phenotype that is reminiscent of hereditary neuropathy with liability to pressure palsies (HNPP), a human disease caused by a 1.5-Mb deletion of a chromosome 17...

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Share to Genetic Predisposition to Disease

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 9143558
DOI: 10.1006/mcne.1997.0600

Genes

PMP22

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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A transgenic mouse model for human hereditary neuropathy with liability to pressure palsies.PMID 9143558Read ↗

Identification of the regulatory region of the peripheral myelin protein 22 (PMP22) gene that directs temporal and spatial expression in development and regeneration of peripheral nerves.Semantic proximity Read ↗

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Correlation between varying levels of PMP22 expression and the degree of demyelination and reduction in nerve conduction velocity in transgenic miceSemantic proximity Read ↗

Stoichiometric alteration of PMP22 protein determines the phenotype of hereditary neuropathy with liability to pressure palsies.Semantic proximity Read ↗

Deletion of the PMP22 gene and hereditary neuropathy with liability to pressure palsies.Semantic proximity Read ↗

Identification of a new Pmp22 mouse mutant and trafficking analysis of a Pmp22 allelic series suggesting that protein aggregates may be protective in Pmp22-associated peripheral neuropathy.Semantic proximity Read ↗

The PMP22 gene and its related diseases.Semantic proximity Read ↗

Heterozygous peripheral myelin protein 22-deficient mice are affected by a progressive demyelinating tomaculous neuropathy.Semantic proximity Read ↗

Pmp22 super-enhancer deletion causes tomacula formation and conduction block in peripheral nerves.Semantic proximity Read ↗

[Molecular genetics of hereditary neuropathies].Semantic proximity Read ↗

Studies on the effects of altered PMP22 expression during myelination in vitroSemantic proximity Read ↗

Hypermyelination and demyelinating peripheral neuropathy in Pmp22-deficient mice.Semantic proximity Read ↗

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