ReviewJournal of anatomy1997
Animal models for inherited peripheral neuropathies.
Review in Journal of anatomy, 1997. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
22 citing papers in PubMed, 1 synthesis or guideline pooled it, 73 citations in OpenAlex.
- Treatment for Charcot-Marie-Tooth disease.The Cochrane database of systematic reviews · 2008Pooled it
- Peripheral Myelin Protein-22 and Its Prominence in Charcot-Marie-Tooth Disease.Chemical reviews · 2026Review
- Article
- Targeting Chemokines and Chemokine GPCRs to Enhance Strong Opioid Efficacy in Neuropathic Pain.Life (Basel, Switzerland) · 2022Review
- Promoting peripheral myelin repair.Experimental neurology · 2016Review
- Curcumin facilitates a transitory cellular stress response in Trembler-J mice.Human molecular genetics · 2013Article
- Defective autoimmune regulator-dependent central tolerance to myelin protein zero is linked to autoimmune peripheral neuropathy.Journal of immunology (Baltimore, Md. : 1950) · 2012Article
- Sox10 is required for Schwann cell identity and progression beyond the immature Schwann cell stage.The Journal of cell biology · 2010Article
- Review
- Article
- Oral curcumin mitigates the clinical and neuropathologic phenotype of the Trembler-J mouse: a potential therapy for inherited neuropathy.American journal of human genetics · 2007Article
- Role of immune cells in animal models for inherited peripheral neuropathies.Neuromolecular medicine · 2006Review
- Loss of Mtmr2 phosphatase in Schwann cells but not in motor neurons causes Charcot-Marie-Tooth type 4B1 neuropathy with myelin outfoldings.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2005Article
- [The role of the immune system in hereditary demyelinating neuropathies].Der Nervenarzt · 2005Review
- Tracing myelin protein zero (P0) in vivo by construction of P0-GFP fusion proteins.BMC cell biology · 2002Article
- Role of immune cells in animal models for inherited neuropathies: facts and visions.Journal of anatomy · 2002Review
- The role of macrophages in demyelinating peripheral nervous system of mice heterozygously deficient in p0.The Journal of cell biology · 2001Article
- Epitope-tagged P(0) glycoprotein causes Charcot-Marie-Tooth-like neuropathy in transgenic mice.The Journal of cell biology · 2000Article
- P(0) glycoprotein overexpression causes congenital hypomyelination of peripheral nerves.The Journal of cell biology · 2000Article
- Immune deficiency in mouse models for inherited peripheral neuropathies leads to improved myelin maintenance.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2000Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
1 author at 1 institution in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Recent progress in human genetics and neurobiology has led to the identification of various mutations in particular myelin genes as the cause for many of the known inherited demyelinating peripheral neuropathies. Mutations in 3 distinct myelin genes, PMP22, P0, and connexin 32 cause the 3 major demyelinating subtypes of Charcot-Marie-Tooth (CMT) disease, CMT1A, CMT1B and CMTX, respectively. In addition, a reduction in the gene dosage of PMP22 causes hereditary neuropathy with liability to pressure palsies (HNPP), while particular point mutations in PMP22 and P0 cause the severe Dejerine-Sottas (DS) neuropathy. A series of spontaneous and genetically engineered rodent mutants for genes for the above-mentioned myelin constituents are now available and their suitability to serve as models for these still untreatable diseases is an issue of particular interest. The spontaneous mutants Trembler-J and Trembler, with point mutations in PMP22, reflect some of the pathological alterations seen in CMT1A and DS patients, respectively. Furthermore, engineered mutants that either over or underexpress particular myelin genes are suitable models for patients who are similarly compromised in the gene dosage of the corresponding genes. In addition, engineered mutants heterozygously or homozygously deficient in the myelin component P0 show the pathology of distinct CMT1B and DS patients, respectively, while Cx32 deficient mice develop pathological abnormalities similar to those of CMTX patients. Mutants that mimic human peripheral neuropathies might allow the development of strategies to alleviate the symptoms of the diseases, and help to define environmental risk factors for aggravation of the disease. In addition, such mutants might be instrumental in the development of strategies to cure the diseases by gene therapy.
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