{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T16:17:31Z","timestamp":1786119451635,"version":"build-2736575974"},"reference-count":15,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2010,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title\/>\n                    <jats:p>Myelin protein zero (MPZ) mutations cause demyelinating neuropathies that range from severe neonatal to milder adult forms. We report a 36-year-old man who developed weakness of his left little finger adduction 3 years earlier. The weakness progressed to his other limbs. Examination revealed mildly high-arched feet with asymmetric weakness of ulnar-innervated muscles (left &gt; right) and asymmetric weakness of peroneal-innervated muscles (right &gt; left). Motor nerve conduction velocities ranged from 18.4 to 24.4 m\/s in the upper extremities and from 14.8 to 22.7 in the lower extremities. Left median partial motor conduction block was noted at the forearm segment. Genetic testing demonstrated MPZ mutation with ARG98HIS amino acid change. The patient's father is a 68-year-old man who was asymptomatic and who was noticed to have high-arched feet and asymmetric leg muscle atrophy and weakness (right &gt; left). The patient's 2-year-old son is \u201cclumsy\u201d with history of neonatal laryngomalacia. He has flat feet, areflexia, and difficulty standing on individual right versus left leg. The patient's paternal grandfather had high-arched feet and hearing loss. We conclude that ARG98HIS MPZ mutation may cause hereditary and relatively mild and asymmetric demyelinating sensorimotor polyneuropathy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1097\/cnd.0b013e3181c5058a","type":"journal-article","created":{"date-parts":[[2010,2,27]],"date-time":"2010-02-27T01:03:34Z","timestamp":1267232614000},"page":"110-113","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":8,"title":["Asymmetric Phenotype Associated With Rare Myelin Protein Zero Mutation"],"prefix":"10.1097","volume":"11","author":[{"given":"Nizar","family":"Souayah","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter Siao","family":"Tick Chong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-2-20240808","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1016\/0092-8674(92)90591-Y","article-title":"Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons.","volume":"71","author":"Giese","year":"1992","journal-title":"Cell"},{"key":"R2-2-20240808","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1083\/jcb.200107114","article-title":"Mutations in the cytoplasmic domain of P0 reveal a role for PKC-mediated phosphorylation in adhesion and myelination.","volume":"155","author":"Xu","year":"2001","journal-title":"J Cell Biol"},{"key":"R3-2-20240808","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/j.jns.2007.05.020","article-title":"Rare myelin protein zero sequence variant in late onset 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nomenclature for PNS myelin proteins.","volume":"4","author":"Eylar","year":"1979","journal-title":"Neurochem Res"},{"key":"R8-2-20240808","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1212\/WNL.59.5.767","article-title":"Corticosteroid- responsive asymmetric neuropathy with a myelin protein zero gene mutation.","volume":"59","author":"Watanabe","year":"2002","journal-title":"Neurology"},{"key":"R9-2-20240808","first-page":"638","article-title":"High frequency of mutations in codon 98 of the peripheral myelin protein P0 gene in 20 French CMT1 patients.","volume":"58","author":"Rouger","year":"1996","journal-title":"Am J Hum Genet"},{"key":"R10-2-20240808","first-page":"546","article-title":"A family of hereditary motor and sensory neuropathy type I with a new type of myelin P0 mutation.","volume":"34","author":"Ohnishi","year":"1994","journal-title":"Rinsho Shinkeigaku"},{"key":"R11-2-20240808","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1136\/jnnp.70.2.232","article-title":"Mild recurrent neuropathy in CMT1B with a novel nonsense mutation in the extracellular domain of the MPZ gene.","volume":"70","author":"Lagueny","year":"2001","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"R12-2-20240808","doi-asserted-by":"crossref","first-page":"1317","DOI":"10.1006\/bbrc.1993.1968","article-title":"Mutation of the myelin P0 gene in Charcot-Marie-tooth neuropathy type 1.","volume":"194","author":"Hayasaka","year":"1993","journal-title":"Biochem Biophys Res Commun"},{"key":"R13-2-20240808","doi-asserted-by":"crossref","first-page":"1369","DOI":"10.1093\/hmg\/2.9.1369","article-title":"Mutation of the myelin P0 gene in Charcot-Marie-Tooth neuropathy type 1B.","volume":"2","author":"Hayasaka","year":"1993","journal-title":"Hum Mol Genet"},{"key":"R14-2-20240808","doi-asserted-by":"crossref","first-page":"1787","DOI":"10.1001\/archneur.63.12.1787","article-title":"Effect of an R69C mutation in the myelin protein zero gene on myelination and ion channel subtypes.","volume":"63","author":"Bai","year":"2006","journal-title":"Arch Neurol"},{"key":"R15-2-20240808","doi-asserted-by":"crossref","first-page":"761","DOI":"10.1212\/WNL.47.3.761","article-title":"Two divergent types of nerve pathology in patients with different P0 mutations in Charcot-Marie-Tooth disease.","volume":"47","author":"Gabreels-Festen","year":"1996","journal-title":"Neurology"}],"container-title":["Journal of Clinical Neuromuscular 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