The Fabry disease-associated lipid lyso-Gb3 enhances voltage-gated calcium currents in sensory neurons and causes pain

Neuroscience Letters

Volumn 594, Issue , 2015, Pages 163-168

  Choi, L ^a   Vernon, J ^a   Kopach, O ^a   Minett, M S ^a   Mills, K ^a,c   Clayton, P T ^a,c   Meert, T ^a,d   Wood, J N ^a,b  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

^b Seoul National University   (South Korea)

^c UNIVERSITY COLLEGE LONDON   (United Kingdom)

^d JANSSEN RESEARCH FOUNDATION   (Belgium)

Author keywords

Calcium imaging; Dorsal root ganglia; Fabry disease; Pain; Voltage dependent Ca2+ channels

Indexed keywords

CALCIUM; CALCIUM CHANNEL; CERAMIDE TRIHEXOSIDE; GLOBOTRIAOSYL LYSOSPHINGOLIPID; GLOBOTRIAOSYLCERAMIDE; GLYCOLIPID; SPHINGOLIPID;

ANIMAL; C57BL MOUSE; CELL CULTURE; CYTOLOGY; DRUG EFFECTS; FABRY DISEASE; HYPERALGESIA; METABOLISM; PAIN; PAIN RECEPTOR; PATHOPHYSIOLOGY; PHYSICAL STIMULATION; PHYSIOLOGY; SPINAL GANGLION; TOUCH;

ANIMALS; CALCIUM; CALCIUM CHANNELS; CELLS, CULTURED; FABRY DISEASE; GANGLIA, SPINAL; GLYCOLIPIDS; HYPERALGESIA; MICE, INBRED C57BL; NOCICEPTORS; PAIN; PHYSICAL STIMULATION; SPHINGOLIPIDS; TOUCH; TRIHEXOSYLCERAMIDES;

EID: 84939979395     PISSN: 03043940     EISSN: 18727972     Source Type: Journal    
DOI: 10.1016/j.neulet.2015.01.084     Document Type: Note

References (20)

1. Fabry H. Angiokeratoma corporis diffusum-Fabry disease: historical review from the original description to the introduction of enzyme replacement therapy. Acta Paediatr. 2002, 91:3-5.
2. Kaye E.M., Kolodny E.H., Logigian E.L., Ullman M.D. Nervous system involvement in Fabry's disease: clinicopathological and biochemical correlation. Ann. Neurol. 1988, 23:505-509.
3. Møller A.T., Jensen T.S. Neurological manifestations in Fabry's disease. Nat. Clin. Pract. Neurol. 2007, 3:95-106.
4. Dütsch M., Marthol H., Stemper B., Brys M., Haendl T., Hilz M.J. Small fiber dysfunction predominates in Fabry neuropathy. J. Clin. Neurophysiol. 2002, 19:575-586.
5. Mills K., Morris P., Lee P., Vellodi A., Waldek S., Young E., Winchester B. Measurement of urinary CDH and CTH by tandem mass spectrometry in patients hemizygous and heterozygous for Fabry disease. J. Inherit. Metab. Dis. 2005, 28:35-48.
6. Young E., Mills K., Morris P., Vellodi A., Lee P., Waldek S., Winchester B. Is globotriaosylceramide a useful biomarker in Fabry disease?. Acta Pediatr. Suppl. 2005, 94:51-54.
7. Aerts J.M., Groener J.E., Kuiper S., Donker-Koopman W.E., Strijland A., Ottenhoff R., van Roomen C., Mirzaian M., Wijburg F.A., Linthorst G.E., Vedder A.C., Rombach S.M., Cox-Brinkman J., Somerharju P., Boot R.G., Hollak C.E., Brady R.O., Poorthuis B.J. Elevated globotriaosylsphingosine is a hallmark of Fabry disease. Proc. Natl. Acad. Sci. U. S. A. 2008, 105:2812-2817.
8. Ohshima T., Murray G.J., Swaim W.D., Longenecker G., Quirk J.M., Cardarelli C.O., Sugimoto Y., Pastan I., Gottesman M.M., Brady R.O., Kulkarni A.B. Alpha-galactosidase A deficient mice: a model of Fabry disease. Proc. Natl. Acad. Sci. U. S. A. 1997, 94:2540-2544.
9. Young-Gqamana B., Brignol N., Chang H.H., Khanna R., Soska R., Fuller M., Sitaraman S.A., Germain D.P., Giugliani R., Hughes D.A., Mehta A., Nicholls K., Boudes P., Lockhart D.J., Valenzano K.J., Benjamin E.R. Migalastat HCl reduces globotriaosylsphingosine (lyso-Gb3) in Fabry transgenic mice and in the plasma of Fabry patients. PLoS One 2013, 8:e57631.
10. Kierkegaard T. Emerging therapies and therapeutic concepts for lysosomal storage diseases. Expert Opin. Orphan Drugs 2013, 1:385-404.
11. Chaplan S.R., Bach F.W., Pogrel J.W., Chung J.M., Yaksh T.L. Quantitative assessment of tactile allodynia in the rat paw. J. Neurosci. Methods 1994, 53:55-63.
12. Minett M.S., Nassar M.A., Clark A.K., Passmore G., Dickenson A.H., Wang F., Malcangio M., Wood J.N. Distinct Nav1.7-dependent pain sensations require different sets of sensory and sympathetic neurons. Nat. Commun. 2012, 3:791.
13. Dupont F.O., Gagnon R., Boutin M., Auray-Blais C. A metabolomic study reveals novel plasma lyso-Gb3 analogs as Fabry disease biomarkers. Curr. Med. Chem. 2013, 20:280-288.
14. Manwaring V., Boutin M., Auray-Blais C. A metabolomic study to identify new globotriaosylceramide-related biomarkers in the plasma of Fabry disease patients. Anal. Chem. 2013, 85:9039-9048.
15. Stucky C.L., Lewin G.R. Isolectin B(4)-positive and -negative nociceptors are functionally distinct. J. Neurosci. 1999, 19:6497-6505.
16. Dirajlal S., Pauers L.E., Stucky C.L. Differential response properties of IB(4)-positive and -negative unmyelinated sensory neurons to protons and capsaicin. J. Neurophysiol. 2003, 89:513-524.
17. Catterall W.A. Voltage-gated calcium channels. Cold Spring Harb. Perspect. Biol. 2011, 3:1-23.
18. Gupta S., Ries M., Kotsopoulos S., Schiffmann R. The relationship of vascular glycolipid storage to clinical manifestations of Fabry disease: a cross-sectional study of a large cohort of clinically affected heterozygous women. Med. (Baltimore) 2005, 84:261-268.
19. Arundine M., Tymianski M. Molecular mechanisms of calcium dependent neurodegeneration in excitotoxicity. Cell Calcium 2003, 34:325-337.
20. Voitenko N.V., Kruglikov I.A., Kostyuk E.P., Kostyuk P.G. Effect of streptozotocin-induced diabetes on the activity of calcium channels in rat dorsal horn neurons. Neuroscience 2000, 95:519-524.