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Voltage\u2010gated sodium channels are responsible for the generation and conduction of action potentials in the peripheral nociceptive neuronal pathway where<jats:styled-content style=\"fixed-case\">Na<jats:sub>V<\/jats:sub>1<\/jats:styled-content>.7,<jats:styled-content style=\"fixed-case\">Na<jats:sub>V<\/jats:sub>1<\/jats:styled-content>.8, and<jats:styled-content style=\"fixed-case\">Na<jats:sub>V<\/jats:sub>1<\/jats:styled-content>.9 sodium channels (encoded by<jats:italic><jats:styled-content style=\"fixed-case\">SCN9A<\/jats:styled-content><\/jats:italic>,<jats:italic><jats:styled-content style=\"fixed-case\">SCN10A<\/jats:styled-content><\/jats:italic>, and<jats:italic><jats:styled-content style=\"fixed-case\">SCN11A<\/jats:styled-content><\/jats:italic>) are preferentially expressed. The human genetic pain conditions inherited erythromelalgia and paroxysmal extreme pain disorder were the first to be linked to gain\u2010of\u2010function<jats:italic><jats:styled-content style=\"fixed-case\">SCN9A<\/jats:styled-content><\/jats:italic>mutations. Recent studies have expanded this spectrum with gain\u2010of\u2010function<jats:italic><jats:styled-content style=\"fixed-case\">SCN9A<\/jats:styled-content><\/jats:italic>mutations in patients with small fiber neuropathy and in a new syndrome of pain, dysautonomia, and small hands and small feet (acromesomelia). In addition, painful neuropathies have been recently linked to<jats:italic><jats:styled-content style=\"fixed-case\">SCN10A<\/jats:styled-content><\/jats:italic>mutations. Patch\u2010clamp studies have shown that the effect of<jats:italic><jats:styled-content style=\"fixed-case\">SCN9A<\/jats:styled-content><\/jats:italic>mutations is dependent upon the cell\u2010type background. The functional effects of a mutation in dorsal root ganglion (<jats:styled-content style=\"fixed-case\">DRG<\/jats:styled-content>) neurons and sympathetic neuron cells may differ per mutation, reflecting the pattern of expression of autonomic symptoms in patients with painful neuropathies who carry the mutation in question. Peripheral neuropathies may not always be length\u2010dependent, as demonstrated in patients with initial facial and scalp pain symptoms with<jats:italic><jats:styled-content style=\"fixed-case\">SCN9A<\/jats:styled-content><\/jats:italic>mutations showing hyperexcitability in both trigeminal ganglion and<jats:styled-content style=\"fixed-case\">DRG<\/jats:styled-content>neurons. There is some evidence suggesting that gain\u2010of\u2010function<jats:italic><jats:styled-content style=\"fixed-case\">SCN9A<\/jats:styled-content><\/jats:italic>mutations can lead to degeneration of peripheral axons. This review will focus on the emerging role of sodium channelopathies in painful peripheral neuropathies, which could serve as a basis for novel therapeutic strategies.<\/jats:p>","DOI":"10.1111\/jns5.12071","type":"journal-article","created":{"date-parts":[[2014,9,23]],"date-time":"2014-09-23T06:10:47Z","timestamp":1411452647000},"page":"53-65","source":"Crossref","is-referenced-by-count":83,"title":["Painful neuropathies: the emerging role of sodium channelopathies"],"prefix":"10.1111","volume":"19","author":[{"given":"Brigitte A.","family":"Brouwer","sequence":"first","affiliation":[{"name":"Department of Anesthesiology and Pain Management Maastricht University Medical Center Maastricht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingemar S. 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