Cargando…

Characterization of small fiber pathology in a mouse model of Fabry disease

Fabry disease (FD) is a life-threatening X-linked lysosomal storage disorder caused by α-galactosidase A (α-GAL) deficiency. Small fiber pathology and pain are major FD symptoms of unknown pathophysiology. α-GAL deficient mice (GLA KO) age-dependently accumulate globotriaosylceramide (Gb3) in dorsal...

Descripción completa

Detalles Bibliográficos
Autores principales:	Hofmann, Lukas, Hose, Dorothea, Grießhammer, Anne, Blum, Robert, Döring, Frank, Dib-Hajj, Sulayman, Waxman, Stephen, Sommer, Claudia, Wischmeyer, Erhard, Üçeyler, Nurcan
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	eLife Sciences Publications, Ltd 2018
Materias:	Human Biology and Medicine
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6255391/ https://www.ncbi.nlm.nih.gov/pubmed/30328411 http://dx.doi.org/10.7554/eLife.39300

Ejemplares similares

Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development
por: Üçeyler, Nurcan, et al.
Publicado: (2016)

Affective and cognitive behavior in the alpha-galactosidase A deficient mouse model of Fabry disease
por: Hofmann, Lukas, et al.
Publicado: (2017)

Dysregulation of Immune Response Mediators and Pain-Related Ion Channels Is Associated with Pain-like Behavior in the GLA KO Mouse Model of Fabry Disease
por: Spitzel, Marlene, et al.
Publicado: (2022)

Differential Impact of miR-21 on Pain and Associated Affective and Cognitive Behavior after Spared Nerve Injury in B7-H1 ko Mouse
por: Karl, Franziska, et al.
Publicado: (2017)

Impaired small fiber conduction in patients with Fabry disease: a neurophysiological case–control study
por: Üçeyler, Nurcan, et al.
Publicado: (2013)

X‐chromosomal inactivation patterns in women with Fabry disease
por: Wagenhäuser, Laura, et al.
Publicado: (2022)

Self-administered version of the Fabry-associated pain questionnaire for adult patients
por: Magg, Barbara, et al.
Publicado: (2015)

English version of the self-administered Fabry Pain Questionnaire for adult patients
por: Jovanovic, Ana, et al.
Publicado: (2020)

Oral Administration of PF-01247324, a Subtype-Selective Nav1.8 Blocker, Reverses Cerebellar Deficits in a Mouse Model of Multiple Sclerosis
por: Shields, Shannon D., et al.
Publicado: (2015)

Skin Globotriaosylceramide 3 Load Is Increased in Men with Advanced Fabry Disease
por: Üçeyler, Nurcan, et al.
Publicado: (2016)

Effects of ranolazine on wild-type and mutant hNa(v)1.7 channels and on DRG neuron excitability
por: Estacion, Mark, et al.
Publicado: (2010)

Multiple myosin motors interact with sodium/potassium-ATPase alpha 1 subunits
por: Dash, Bhagirathi, et al.
Publicado: (2018)

Increased Arterial Diameters in the Posterior Cerebral Circulation in Men with Fabry Disease
por: Üçeyler, Nurcan, et al.
Publicado: (2014)

Mutation I136V alters electrophysiological properties of the Na(V)1.7 channel in a family with onset of erythromelalgia in the second decade
por: Cheng, Xiaoyang, et al.
Publicado: (2008)

Nonmuscle myosin II isoforms interact with sodium channel alpha subunits
por: Dash, Bhagirathi, et al.
Publicado: (2018)

Expression of Nav1.7 in DRG neurons extends from peripheral terminals in the skin to central preterminal branches and terminals in the dorsal horn
por: Black, Joel A, et al.
Publicado: (2012)

Brain activity associated with pain in inherited erythromelalgia: stimulus-free pain engages brain areas involved in valuation and learning
por: Geha, Paul, et al.
Publicado: (2018)

Kv7-specific activators hyperpolarize resting membrane potential and modulate human iPSC-derived sensory neuron excitability
por: Estacion, Mark, et al.
Publicado: (2023)

The fates of internalized Na(V)1.7 channels in sensory neurons: Retrograde cotransport with other ion channels, axon-specific recycling, and degradation
por: Higerd-Rusli, Grant P., et al.
Publicado: (2022)

Cytokine expression profiles in white blood cells of patients with small fiber neuropathy
por: Kreß, Luisa, et al.
Publicado: (2023)

Capsaicin 8% patch reversibly reduces A-delta fiber evoked potential amplitudes
por: Papagianni, Aikaterini, et al.
Publicado: (2018)

Organ manifestations and long-term outcome of Fabry disease in patients with the GLA haplotype D313Y
por: Oder, Daniel, et al.
Publicado: (2016)

Conserved but not critical: Trafficking and function of Na(V)1.7 are independent of highly conserved polybasic motifs
por: Tyagi, Sidharth, et al.
Publicado: (2023)

Multicenter Female Fabry Study (MFFS) - clinical survey on current treatment of females with Fabry disease
por: Lenders, Malte, et al.
Publicado: (2016)

Inflammation differentially controls transport of depolarizing Nav versus hyperpolarizing Kv channels to drive rat nociceptor activity
por: Higerd-Rusli, Grant P., et al.
Publicado: (2023)

Gene variants of unknown significance in Fabry disease: Clinical characteristics of c.376A>G (p.Ser126Gly)
por: Lau, Kolja, et al.
Publicado: (2022)

Preferential Targeting of Na(v)1.6 Voltage-Gated Na(+) Channels to the Axon Initial Segment during Development
por: Akin, Elizabeth J., et al.
Publicado: (2015)

Patient‐derived in vitro skin models for investigation of small fiber pathology
por: Karl, Franziska, et al.
Publicado: (2019)

Paclitaxel increases axonal localization and vesicular trafficking of Na(v)1.7
por: Akin, Elizabeth J, et al.
Publicado: (2021)

Conditional knockout of Na(V)1.6 in adult mice ameliorates neuropathic pain
por: Chen, Lubin, et al.
Publicado: (2018)

Paclitaxel effects on axonal localization and vesicular trafficking of Na(V)1.8
por: Baker, Christopher A., et al.
Publicado: (2023)

Activation of TRESK channels by the inflammatory mediator lysophosphatidic acid balances nociceptive signalling
por: Kollert, Sina, et al.
Publicado: (2015)

A novel gain-of-function Na(v)1.7 mutation in a carbamazepine-responsive patient with adult-onset painful peripheral neuropathy
por: Adi, Talia, et al.
Publicado: (2018)

Non-psychotropic phytocannabinoid interactions with voltage-gated sodium channels: An update on cannabidiol and cannabigerol
por: Ghovanloo, Mohammad-Reza, et al.
Publicado: (2022)

Amplitudes of Pain-Related Evoked Potentials Are Useful to Detect Small Fiber Involvement in Painful Mixed Fiber Neuropathies in Addition to Quantitative Sensory Testing – An Electrophysiological Study
por: Hansen, Niels, et al.
Publicado: (2015)

A Novel Gain-of-Function Nav1.9 Mutation in a Child With Episodic Pain
por: Huang, Jianying, et al.
Publicado: (2019)

Mutations at opposite ends of the DIII/S4-S5 linker of sodium channel Na(V)1.7 produce distinct pain disorders
por: Cheng, Xiaoyang, et al.
Publicado: (2010)

Na(v)1.7 is the predominant sodium channel in rodent olfactory sensory neurons
por: Ahn, Hye-Sook, et al.
Publicado: (2011)

The AMPK Activator A769662 Blocks Voltage-Gated Sodium Channels: Discovery of a Novel Pharmacophore with Potential Utility for Analgesic Development
por: Asiedu, Marina N., et al.
Publicado: (2017)

Genomic analysis of 21 patients with corneal neuralgia after refractive surgery
por: Yuan, Jun-Hui, et al.
Publicado: (2020)

Cannot write session to /tmp/vufind_sessions/sess_232cso017bf4jp73mpirliq3m7