Sulayman D Dib-Hajj

Summary

Affiliation: Yale University
Country: USA

Publications

  1. pmc Temperature dependence of erythromelalgia mutation L858F in sodium channel Nav1.7
    Chongyang Han
    Institute of Materia Medica, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100050, China
    Mol Pain 3:3. 2007
  2. doi request reprint Isoform-specific and pan-channel partners regulate trafficking and plasma membrane stability; and alter sodium channel gating properties
    Sulayman D Dib-Hajj
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, United States
    Neurosci Lett 486:84-91. 2010
  3. doi request reprint Voltage-gated sodium channels in pain states: role in pathophysiology and targets for treatment
    Sulayman D Dib-Hajj
    Department of Neurology and Center for Neuroscience and Regeneration Research Yale University School of Medicine, New Haven, CT 06510, USA
    Brain Res Rev 60:65-83. 2009
  4. pmc Paroxysmal extreme pain disorder M1627K mutation in human Nav1.7 renders DRG neurons hyperexcitable
    Sulayman D Dib-Hajj
    Deptartment of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Mol Pain 4:37. 2008
  5. ncbi request reprint Fibroblast growth factor homologous factor 2B: association with Nav1.6 and selective colocalization at nodes of Ranvier of dorsal root axons
    Ellen K Wittmack
    Department of Pharmacology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 24:6765-75. 2004
  6. ncbi request reprint Voltage-gated sodium channel Nav1.6 is modulated by p38 mitogen-activated protein kinase
    Ellen K Wittmack
    Department of Pharmacology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 25:6621-30. 2005
  7. doi request reprint Sodium channel Na(v)1.7 is essential for lowering heat pain threshold after burn injury
    Shannon D Shields
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06520, USA
    J Neurosci 32:10819-32. 2012
  8. pmc Two Nedd4-binding motifs underlie modulation of sodium channel Nav1.6 by p38 MAPK
    Andreas Gasser
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Biol Chem 285:26149-61. 2010
  9. doi request reprint Phosphorylation of sodium channel Na(v)1.8 by p38 mitogen-activated protein kinase increases current density in dorsal root ganglion neurons
    Andy Hudmon
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 28:3190-201. 2008
  10. doi request reprint A sodium channel gene SCN9A polymorphism that increases nociceptor excitability
    Mark Estacion
    Department of Neurology, Yale University School of Medicine, New Haven CT
    Ann Neurol 66:862-6. 2009

Collaborators

Detail Information

Publications74

  1. pmc Temperature dependence of erythromelalgia mutation L858F in sodium channel Nav1.7
    Chongyang Han
    Institute of Materia Medica, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100050, China
    Mol Pain 3:3. 2007
    ..It has recently been shown that several disease-causing erythromelalgia mutations alter channel-gating behavior in a manner that increases DRG neuron excitability...
  2. doi request reprint Isoform-specific and pan-channel partners regulate trafficking and plasma membrane stability; and alter sodium channel gating properties
    Sulayman D Dib-Hajj
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, United States
    Neurosci Lett 486:84-91. 2010
    ..Here we review the important functional role of pan-sodium channel and isoform-specific partners in regulating sodium current density and gating properties...
  3. doi request reprint Voltage-gated sodium channels in pain states: role in pathophysiology and targets for treatment
    Sulayman D Dib-Hajj
    Department of Neurology and Center for Neuroscience and Regeneration Research Yale University School of Medicine, New Haven, CT 06510, USA
    Brain Res Rev 60:65-83. 2009
    ..9 as particularly important in the pathophysiology of different pain syndromes, and isoform-specific blockers of these channels or targeting their modulators hold the promise of a future effective therapy for treatment of pain...
  4. pmc Paroxysmal extreme pain disorder M1627K mutation in human Nav1.7 renders DRG neurons hyperexcitable
    Sulayman D Dib-Hajj
    Deptartment of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Mol Pain 4:37. 2008
    ..Carbamazepine has been effective in relieving symptoms, while other drugs including other anti-epileptics are less effective...
  5. ncbi request reprint Fibroblast growth factor homologous factor 2B: association with Nav1.6 and selective colocalization at nodes of Ranvier of dorsal root axons
    Ellen K Wittmack
    Department of Pharmacology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 24:6765-75. 2004
    ..The preferential expression of FHF2B in sensory neurons may provide a basis for physiological differences in sodium currents that have been reported at the nodes of Ranvier in sensory versus motor axons...
  6. ncbi request reprint Voltage-gated sodium channel Nav1.6 is modulated by p38 mitogen-activated protein kinase
    Ellen K Wittmack
    Department of Pharmacology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 25:6621-30. 2005
    ..6 current density. This is the first demonstration of MAPK phosphorylation and modulation of a voltage-gated sodium channel, and this modulation may represent an additional role for MAPK in regulating the neuronal response to injury...
  7. doi request reprint Sodium channel Na(v)1.7 is essential for lowering heat pain threshold after burn injury
    Shannon D Shields
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06520, USA
    J Neurosci 32:10819-32. 2012
    ..Our results offer insights into the molecular and cellular mechanisms of modality-specific pain signaling, and suggest Na(v)1.7-blocking drugs may be effective in burn patients...
  8. pmc Two Nedd4-binding motifs underlie modulation of sodium channel Nav1.6 by p38 MAPK
    Andreas Gasser
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Biol Chem 285:26149-61. 2010
    ..6 is necessary for stress-induced current modulation, with positive or negative regulation depending upon the availability of the C-terminal Pro-Ser-Tyr motif to bind Nedd4-2...
  9. doi request reprint Phosphorylation of sodium channel Na(v)1.8 by p38 mitogen-activated protein kinase increases current density in dorsal root ganglion neurons
    Andy Hudmon
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 28:3190-201. 2008
    ..Our study suggests a mechanism by which activated p38 contributes to inflammatory, and possibly neuropathic, pain through a p38-mediated increase of Na(v)1.8 current density...
  10. doi request reprint A sodium channel gene SCN9A polymorphism that increases nociceptor excitability
    Mark Estacion
    Department of Neurology, Yale University School of Medicine, New Haven CT
    Ann Neurol 66:862-6. 2009
    ..Our results suggest that polymorphisms in the Na(V)1.7 channel may influence susceptibility to pain...
  11. ncbi request reprint Contactin associates with sodium channel Nav1.3 in native tissues and increases channel density at the cell surface
    Bhaval S Shah
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 24:7387-99. 2004
    ..We propose that the upregulation of contactin and its colocalization with Na(v)1.3 in axotomized DRG neurons may contribute to the hyper-excitablity of the injured neurons...
  12. doi request reprint Small-fiber neuropathy Nav1.8 mutation shifts activation to hyperpolarized potentials and increases excitability of dorsal root ganglion neurons
    Jianying Huang
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 33:14087-97. 2013
    ....
  13. doi request reprint Functional profiles of SCN9A variants in dorsal root ganglion neurons and superior cervical ganglion neurons correlate with autonomic symptoms in small fibre neuropathy
    Chongyang Han
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Brain 135:2613-28. 2012
    ....
  14. doi request reprint Deletion mutation of sodium channel Na(V)1.7 in inherited erythromelalgia: enhanced slow inactivation modulates dorsal root ganglion neuron hyperexcitability
    Xiaoyang Cheng
    Department of Neurology and Centre for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT, USA
    Brain 134:1972-86. 2011
    ..7 channels. Our results suggest that despite the pivotal role of activation shift in inherited erythromelalgia development, slow inactivation may regulate clinical phenotype by altering channel availability...
  15. ncbi request reprint Inherited erythermalgia: limb pain from an S4 charge-neutral Na channelopathy
    Jin Sung Choi
    Department of Neurology, Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT 06510, USA
    Neurology 67:1563-7. 2006
    ..7 sodium channel, which is preferentially expressed in nociceptors. Thus far, Na(v)1.7 mutations within intracellular linker parts of the channel have been physiologically characterized...
  16. ncbi request reprint Size matters: Erythromelalgia mutation S241T in Nav1.7 alters channel gating
    Angelika Lampert
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Biol Chem 281:36029-35. 2006
    ..We conclude that the linker between S4 and S5 in domain I of Nav1.7 modulates gating of this channel, and that a larger side chain at position 241 interferes with its gating mechanisms...
  17. doi request reprint Erythromelalgia mutation L823R shifts activation and inactivation of threshold sodium channel Nav1.7 to hyperpolarized potentials
    Angelika Lampert
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Biochem Biophys Res Commun 390:319-24. 2009
    ..7 leads to a pronounced hyperpolarizing shift of activation, a change that is expected to increase nociceptor excitability despite the hyperpolarizing shift in fast-inactivation, which is unique among the IEM mutations...
  18. pmc Distinct repriming and closed-state inactivation kinetics of Nav1.6 and Nav1.7 sodium channels in mouse spinal sensory neurons
    Raimund I Herzog
    Department of Neurology and PVA EPVA Neuroscience Research Center, Yale Medical School, New Haven, CT 06510, USA
    J Physiol 551:741-50. 2003
    ..7 currents. Our results indicate that the firing properties of DRG neurons can be tuned by regulating expression of different sodium channel isoforms that have distinct repriming and closed-state inactivation kinetics...
  19. ncbi request reprint Contactin regulates the current density and axonal expression of tetrodotoxin-resistant but not tetrodotoxin-sensitive sodium channels in DRG neurons
    Anthony M Rush
    Department of Neurology, Yale School of Medicine, LCI 707, 333 Cedar St, New Haven, CT 06510, USA
    Eur J Neurosci 22:39-49. 2005
    ....
  20. pmc Electrophysiological properties of two axonal sodium channels, Nav1.2 and Nav1.6, expressed in mouse spinal sensory neurones
    Anthony M Rush
    Department of Neurology, Center for Neuroscience and Regeneration Research, Yale School of Medicine, LCI 707, 333 Cedar Street, New Haven, CT 06510, USA
    J Physiol 564:803-15. 2005
    ..6 and the Na(+)/Ca(2+) exchanger are colocalized, while selective expression of Na(v)1.2 may support action potential electrogenesis, at least at lower frequencies, while producing a smaller persistent current...
  21. ncbi request reprint Calmodulin regulates current density and frequency-dependent inhibition of sodium channel Nav1.8 in DRG neurons
    Jin Sung Choi
    Department of Neurology, Yale University School of Medicine, New Heaven, CT, USA
    J Neurophysiol 96:97-108. 2006
    ..8, in native neurons, and demonstrates a regulation of Nav1.8 currents that can significantly affect electrogenesis of DRG neurons in which Nav1.8 is normally expressed...
  22. doi request reprint ERK1/2 mitogen-activated protein kinase phosphorylates sodium channel Na(v)1.7 and alters its gating properties
    Severine Stamboulian
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 30:1637-47. 2010
    ..7 by pERK1/2, which unlike the modulation of Na(v)1.6 and Na(v)1.8 by pp38, regulates gating properties of this channel but not its current density and contributes to the effects of MAPKs on DRG neuron excitability...
  23. ncbi request reprint Differential modulation of sodium channel Na(v)1.6 by two members of the fibroblast growth factor homologous factor 2 subfamily
    Anthony M Rush
    Department of Neurology, Yale School of Medicine, New Haven, CT 06510, USA
    Eur J Neurosci 23:2551-62. 2006
    ..6 and are differentially distributed in DRG neurons and their axons. This suggests that FHF2A and FHF2B may selectively alter firing behaviour of specific neuronal compartments via differential modulation of Na(v)1.6...
  24. doi request reprint Alternative splicing may contribute to time-dependent manifestation of inherited erythromelalgia
    Jin Sung Choi
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Brain 133:1823-35. 2010
    ..This study shows that a change in relative expression of splice isoforms can contribute to time-dependent manifestation of the functional phenotype of a sodium channelopathy...
  25. doi request reprint Mexiletine-responsive erythromelalgia due to a new Na(v)1.7 mutation showing use-dependent current fall-off
    Jin Sung Choi
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Exp Neurol 216:383-9. 2009
    ..7 channels. Continued relief from pain, even after mexiletine was discontinued in this patient, might suggest that early treatment may slow the progression of the disease...
  26. pmc Mutations at opposite ends of the DIII/S4-S5 linker of sodium channel Na V 1.7 produce distinct pain disorders
    Xiaoyang Cheng
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT, USA
    Mol Pain 6:24. 2010
    ....
  27. pmc Mutation I136V alters electrophysiological properties of the Na(v)1.7 channel in a family with onset of erythromelalgia in the second decade
    Xiaoyang Cheng
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    Mol Pain 4:1. 2008
    ..7 mutations reported to date, which have been localized in the voltage sensor S4, the linker joining segments S4 and S5 or pore-lining segments S5 and S6 in DI, II and III...
  28. ncbi request reprint Differential slow inactivation and use-dependent inhibition of Nav1.8 channels contribute to distinct firing properties in IB4+ and IB4- DRG neurons
    Jin Sung Choi
    Department of Neurology, Yale School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA
    J Neurophysiol 97:1258-65. 2007
    ..8 current in these two DRG subpopulations, which results from their different rate of entry into and recovery from the slow inactivation state, contributes to functional differences between these two neuronal populations...
  29. pmc Intra- and interfamily phenotypic diversity in pain syndromes associated with a gain-of-function variant of NaV1.7
    Mark Estacion
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Mol Pain 7:92. 2011
    ..In this paper we describe three patients who house the NaV1.7/I228M variant...
  30. doi request reprint A channelopathy contributes to cerebellar dysfunction in a model of multiple sclerosis
    Shannon D Shields
    Department of Neurology, Yale University School of Medicine, New Haven, CT, USA
    Ann Neurol 71:186-94. 2012
    ..Here, we tested the hypothesis that upregulation of Nav1.8 in cerebellum in MS and EAE has functional consequences contributing to symptom burden...
  31. ncbi request reprint Patterned electrical activity modulates sodium channel expression in sensory neurons
    Joshua P Klein
    Department of Neurology and PVA EPVA Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci Res 74:192-8. 2003
    ..These results show that a change in neuronal activity can alter the expression of sodium channel genes in a subtype-specific manner, via a mechanism independent of NGF withdrawal...
  32. ncbi request reprint Na(V)1.7 mutant A863P in erythromelalgia: effects of altered activation and steady-state inactivation on excitability of nociceptive dorsal root ganglion neurons
    T Patrick Harty
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 26:12566-75. 2006
    ..Thus, A863P mutant channels produce hyperexcitability in DRG neurons, which contributes to the pathophysiology of IEM...
  33. doi request reprint A sodium channel mutation linked to epilepsy increases ramp and persistent current of Nav1.3 and induces hyperexcitability in hippocampal neurons
    Mark Estacion
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Exp Neurol 224:362-8. 2010
    ..Our data provide a pathophysiological basis for the pathogenicity of the first epilepsy-linked mutation within Na(V)1.3 channels and hippocampal neurons...
  34. doi request reprint Transfection of rat or mouse neurons by biolistics or electroporation
    Sulayman D Dib-Hajj
    Department of Neurology, School of Medicine, Yale University, New Haven, CT, USA
    Nat Protoc 4:1118-26. 2009
    ..Although we have used sodium channels for the examples that we show here, these methods can also be used to study other types of molecules...
  35. doi request reprint A new Nav1.7 mutation in an erythromelalgia patient
    Mark Estacion
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Biochem Biophys Res Commun 432:99-104. 2013
    ..9 mV), which is predicted to attenuate the effect of this mutation on DRG neuron firing. These changes are consistent with previously characterized Erytheromelalgia associated mutations of Nav1.7...
  36. doi request reprint The Na(V)1.7 sodium channel: from molecule to man
    Sulayman D Dib-Hajj
    Department of Neurology, and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    Nat Rev Neurosci 14:49-62. 2013
    ..7 is a major contributor to pain signalling in humans, and homology modelling based on crystal structures of ion channels suggests an atomic-level structural basis for the altered gating of mutant Na(V)1.7 that causes pain...
  37. pmc Structural modelling and mutant cycle analysis predict pharmacoresponsiveness of a Na(V)1.7 mutant channel
    Yang Yang
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    Nat Commun 3:1186. 2012
    ..We suggest that this approach might identify variants that confer enhanced pharmacoresponsiveness on a variety of channels...
  38. pmc An ankyrinG-binding motif is necessary and sufficient for targeting Nav1.6 sodium channels to axon initial segments and nodes of Ranvier
    Andreas Gasser
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Neurosci 32:7232-43. 2012
    ..Thus, the ankyrinG-binding motif is both necessary and sufficient for the clustering of sodium channels at nodes of Ranvier and the AIS...
  39. pmc PKCε phosphorylation of the sodium channel NaV1.8 increases channel function and produces mechanical hyperalgesia in mice
    Dai Fei Wu
    Ernest Gallo Clinic and Research Center, Department of Neurology, UCSF, Emeryville, California 94608, USA
    J Clin Invest 122:1306-15. 2012
    ..8 channels. These studies demonstrate that NaV1.8 is an important, direct substrate of PKCε that mediates PKCε-dependent mechanical hyperalgesia...
  40. pmc Nav1.7 is the predominant sodium channel in rodent olfactory sensory neurons
    Hye Sook Ahn
    Department of Neurology, Yale University School of Medicine, New Haven, 06520, USA
    Mol Pain 7:32. 2011
    ..These findings led us to hypothesize that Nav1.7 is the main sodium channel in the peripheral olfactory sensory neurons (OSN, also known as olfactory receptor neurons)...
  41. ncbi request reprint CAP-1A is a novel linker that binds clathrin and the voltage-gated sodium channel Na(v)1.8
    Chuanju Liu
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Mol Cell Neurosci 28:636-49. 2005
    ..CAP-1A thus is the first example of an adapter protein that links clathrin and a sodium channel and may regulate Na(v)1.8 channel density at the cell surface...
  42. doi request reprint Early- and late-onset inherited erythromelalgia: genotype-phenotype correlation
    Chongyang Han
    Department of Neurology, LCI 707, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06520 8018, USA
    Brain 132:1711-22. 2009
    ....
  43. ncbi request reprint Characterization and developmental changes of Na+ currents of petrosal neurons with projections to the carotid body
    Theodore R Cummins
    Department of Neurology, Yale University School of Medicine, New Haven Connecticut 06510, USA
    J Neurophysiol 88:2993-3002. 2002
    ....
  44. ncbi request reprint A case of inherited erythromelalgia
    Steven P Novella
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06520 8018, USA
    Nat Clin Pract Neurol 3:229-34. 2007
    ..The episodes were triggered by heat or exertion. His medical history revealed an extensive six-generation family history of similar symptoms...
  45. doi request reprint A new Nav1.7 sodium channel mutation I234T in a child with severe pain
    Hye Sook Ahn
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Eur J Pain 14:944-50. 2010
    ..Although enhanced slow-inactivation may attenuate the gain-of-function of the I234T mutation, the shift in activation appears to be dominant, and is consistent with the severe pain symptoms reported in this patient...
  46. pmc Molecular architecture of a sodium channel S6 helix: radial tuning of the voltage-gated sodium channel 1.7 activation gate
    Yang Yang
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Biol Chem 288:13741-7. 2013
    ..In-frame deletion mutation (Del-L955) in NaV1.7 sodium channel from a kindred with erythromelalgia hyperpolarizes activation...
  47. doi request reprint Nav1.8 expression is not restricted to nociceptors in mouse peripheral nervous system
    Shannon D Shields
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT, USA
    Pain 153:2017-30. 2012
    ..Our results indicate that Na(v)1.8 underlies electrical activity of sensory neurons subserving multiple functional modalities, and call for cautious interpretation of the phenotypes of Na(v)1.8-Cre-driven conditional knockout mice...
  48. pmc A pore-blocking hydrophobic motif at the cytoplasmic aperture of the closed-state Nav1.7 channel is disrupted by the erythromelalgia-associated F1449V mutation
    Angelika Lampert
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT 06510, USA
    J Biol Chem 283:24118-27. 2008
    ..We propose that the four aromatic residues contribute to the gate at the cytoplasmic pore aperture, and that their ring side chains form a hydrophobic plug which stabilizes the closed state of Na(v)1.7...
  49. doi request reprint A novel Nav1.7 mutation producing carbamazepine-responsive erythromelalgia
    Tanya Z Fischer
    Department of Neurology, Yale University School of Medicine, New Haven, CT 16510, USA
    Ann Neurol 65:733-41. 2009
    ..Inherited erythromelalgia (IEM) has been linked to gain-of-function mutations of Na(v)1.7. We now report a novel mutation (V400M) in a three-generation Canadian family in which pain is relieved by carbamazepine (CBZ)...
  50. ncbi request reprint Modulation of the cardiac sodium channel Nav1.5 by fibroblast growth factor homologous factor 1B
    Chuan Ju Liu
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Biol Chem 278:1029-36. 2003
    ..5 channel with FHF1B. This is the first report showing that interaction with a growth factor can modulate properties of a voltage-gated sodium channel...
  51. pmc A single sodium channel mutation produces hyper- or hypoexcitability in different types of neurons
    Anthony M Rush
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT 06510, USA
    Proc Natl Acad Sci U S A 103:8245-50. 2006
    ..Moreover, these findings show that a single ion channel mutation can produce opposing phenotypes (hyperexcitability or hypoexcitability) in the different cell types in which the channel is expressed...
  52. ncbi request reprint Structure of the sodium channel gene SCN11A: evidence for intron-to-exon conversion model and implications for gene evolution
    Sulayman D Dib-Hajj
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Mol Neurobiol 26:235-50. 2002
    ..Although a functional role for Nav1.9b has yet to be established, intron-to-exon conversion may represent a mechanism for ion channels to acquire novel features...
  53. ncbi request reprint From genes to pain: Na v 1.7 and human pain disorders
    Sulayman D Dib-Hajj
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Trends Neurosci 30:555-63. 2007
    ..The contribution of Na(v)1.7 to acquired and inherited pain states and the absence of motor, cognitive and cardiac deficits in patients lacking this channel make it an attractive target for the treatment of neuropathic pain...
  54. doi request reprint Voltage-gated sodium channels: therapeutic targets for pain
    Sulayman D Dib-Hajj
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT 06520 8018, USA
    Pain Med 10:1260-9. 2009
    ..To provide an overview of the role of voltage-gated sodium channels in pathophysiology of acquired and inherited pain states, and of recent developments that validate these channels as therapeutic targets for treating chronic pain...
  55. doi request reprint Differential effect of D623N variant and wild-type Na(v)1.7 sodium channels on resting potential and interspike membrane potential of dorsal root ganglion neurons
    Hye Sook Ahn
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510 8018, USA
    Brain Res 1529:165-77. 2013
    ..7 associated with painful neuropathy depolarizes resting membrane potential and produces an enhanced inward current during interspike intervals, thereby contributing to DRG neuron hyperexcitability. ..
  56. pmc Virus-mediated shRNA knockdown of Na(v)1.3 in rat dorsal root ganglion attenuates nerve injury-induced neuropathic pain
    Omar A Samad
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut, USA
    Mol Ther 21:49-56. 2013
    ..Taken together, our studies support the contribution of peripheral Na(v)1.3 to pain in adult rats with neuropathic pain, validate Na(v)1.3 as a target, and provide validation for this approach of AAV-mediated peripheral gene therapy...
  57. doi request reprint Sodium channels in normal and pathological pain
    Sulayman D Dib-Hajj
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    Annu Rev Neurosci 33:325-47. 2010
    ..Na(v)1.7 is of special interest because it has been linked to a spectrum of inherited human pain disorders. Here we review the contribution of these sodium channel isoforms to pain...
  58. doi request reprint Multistate structural modeling and voltage-clamp analysis of epilepsy/autism mutation Kv10.2-R327H demonstrate the role of this residue in stabilizing the channel closed state
    Yang Yang
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, Connecticut 06510, Rehabilitation Research Center, Veterans Affairs Connecticut Healthcare System, West Haven, Connecticut 06516, and Arizona Research Laboratories Division of Biotechnology, University of Arizona, Tucson, Arizona 85721
    J Neurosci 33:16586-93. 2013
    ..2 channel mutation associated with neurological disease. ..
  59. pmc Expression of Nav1.7 in DRG neurons extends from peripheral terminals in the skin to central preterminal branches and terminals in the dorsal horn
    Joel A Black
    Department of Neurology and Paralyzed Veterans of America Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT 06510, USA
    Mol Pain 8:82. 2012
    ..7 in rat dorsal root ganglia neurons, from peripheral terminals in the skin to central terminals in the spinal cord dorsal horn...
  60. ncbi request reprint Functional role of the C-terminus of voltage-gated sodium channel Na(v)1.8
    Jin Sung Choi
    Department of Neurology, Yale University School of Medicine, New Haven CT 06510, USA
    FEBS Lett 572:256-60. 2004
    ..4/1.8C currents demonstrate a non-inactivated fraction. Thus, the C-terminus of Na(v)1.8 contributes to regulation of channel density at the cell surface, modulates channel gating, and regulates the generation of sustained current...
  61. doi request reprint Genetics and molecular pathophysiology of Na(v)1.7-related pain syndromes
    Sulayman D Dib-Hajj
    Department of Neurology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    Adv Genet 63:85-110. 2008
    ..The central role of Na(v)1.7 in these disorders, and the apparently limited consequences of loss of this channel in humans make it an attractive target for treatment of pain...
  62. pmc Effects of ranolazine on wild-type and mutant hNav1.7 channels and on DRG neuron excitability
    Mark Estacion
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Mol Pain 6:35. 2010
    ..7 and on DRG neuron excitability have not been investigated. We used voltage- and current-clamp recordings to evaluate the hypothesis that ranolazine may be effective in regulating Nav1.7-induced DRG neuron hyperexcitability...
  63. doi request reprint Voltage-gated sodium channel expression in rat and human epidermal keratinocytes: evidence for a role in pain
    Peng Zhao
    Department of Neurology and Center for Neuroscience and Regeneration Research, Yale University School of Medicine, New Haven, CT 06510, USA
    Pain 139:90-105. 2008
    ....
  64. ncbi request reprint Erythromelalgia: a hereditary pain syndrome enters the molecular era
    Stephen G Waxman
    Department of Neurology, Yale University School of Medicine, New Haven, CT 06510, USA
    Ann Neurol 57:785-8. 2005
    ....
  65. ncbi request reprint Critical molecular determinants of voltage-gated sodium channel sensitivity to mu-conotoxins GIIIA/B
    Theodore R Cummins
    Department of Neurology and PVA EPVA Neuroscience Research Center, Yale University School of Medicine, New Haven, Connecticut 06516, USA
    Mol Pharmacol 61:1192-201. 2002
    ..Our results should aid the development of toxins that block specific neuronal sodium channel isoforms...
  66. pmc Tetrodotoxin-sensitive Na+ channels and muscarinic and purinergic receptors identified in human erythroid progenitor cells and red blood cell ghosts
    Joseph F Hoffman
    Department of Cellular and Molecular Physiology, Yale University School of Medicine, New Haven, CT 06520, USA
    Proc Natl Acad Sci U S A 101:12370-4. 2004
    ..Implications regarding the presence of these different types of channels and receptors in human red blood cells and their functional significance are discussed...
  67. ncbi request reprint Electrophysiological properties of mutant Nav1.7 sodium channels in a painful inherited neuropathy
    Theodore R Cummins
    Department of Pharmacology and Toxicology, Stark Neurosciences Research Institute, Indiana University School of Medicine, Indianapolis, Indiana 46202, USA
    J Neurosci 24:8232-6. 2004
    ....
  68. ncbi request reprint Nav1.6 channels generate resurgent sodium currents in spinal sensory neurons
    Theodore R Cummins
    Department of Pharmacology and Toxicology, Stark Neurosciences Research Institute, Indiana University School of Medicine, Indianapolis, IN 46202, USA
    FEBS Lett 579:2166-70. 2005
    ..6 produce resurgent currents. These results demonstrate for the first time the intrinsic ability of Na(v)1.6 to produce a resurgent current, and also show that cell background is critical in permitting the generation of these currents...
  69. ncbi request reprint Sporadic onset of erythermalgia: a gain-of-function mutation in Nav1.7
    Chongyang Han
    Institute of Materia Medica, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
    Ann Neurol 59:553-8. 2006
    ..We investigated the role of Na(v)1.7 in a sporadic case of erythermalgia in a Chinese family...
  70. ncbi request reprint Pharmacological properties of neuronal TTX-resistant sodium channels and the role of a critical serine pore residue
    Andreas Leffler
    Klinik fur Anasthesiologie, Friedrich Alexander Universität Erlangen Nuremberg, Krankenhausstr 12, 91054 Erlangen, Germany
    Pflugers Arch 451:454-63. 2005
    ....
  71. pmc A Nav1.7 channel mutation associated with hereditary erythromelalgia contributes to neuronal hyperexcitability and displays reduced lidocaine sensitivity
    Patrick L Sheets
    Department of Pharmacology and Toxicology, Stark Neurosciences Research Institute, Indiana University School of Medicine, 950 West Walnut St, R2 468, Indianapolis, IN 46202, USA
    J Physiol 581:1019-31. 2007
    ..7 and suggests that the response of individuals with hereditary erythromelalgia to lidocaine treatment may be determined, at least in part, by their specific genotype...
  72. ncbi request reprint Molecular determinant of Na(v)1.8 sodium channel resistance to the venom from the scorpion Leiurus quinquestriatus hebraeus
    Carl Y Saab
    Department of Neurology and PVA EPVA Neuroscience Research Center, Yale Medical School, CT New Haven 06510, USA
    Neurosci Lett 331:79-82. 2002
    ..Therefore, we conclude that the tetrapeptide SLEN at the D4S3-S4 linker region is sufficient to make Na(v)1.8 resistant to LQTX...
  73. ncbi request reprint The presence and role of the tetrodotoxin-resistant sodium channel Na(v)1.9 (NaN) in nociceptive primary afferent neurons
    Xin Fang
    Department of Physiology, University of Bristol, Medical School, Bristol BS8 1TD, United Kingdom
    J Neurosci 22:7425-33. 2002
    ..The data provide direct evidence that Na(v)1.9 is expressed selectively in (but not in all) C- and A-fiber nociceptive-type units and suggest that Na(v)1.9 contributes to membrane properties that are typical of nociceptive neurons...
  74. ncbi request reprint GDNF and NGF reverse changes in repriming of TTX-sensitive Na(+) currents following axotomy of dorsal root ganglion neurons
    Andreas Leffler
    Department of Neurology and Paralyzed Veterans of America Eastern Paralyzed Veterans Association Neuroscience Research Center, Yale Medical School, New Haven 06510, CT, USA
    J Neurophysiol 88:650-8. 2002
    ..3 mRNA, with GDNF plus NGF producing the largest effect. Our data indicate that both GDNF and NGF can partially reverse an important effect of axotomy on the electrogenic properties of sensory neurons and that their effect is additive...