Delphine Bouhy

Delphine Bouhy
Peripheral Neuropathy Group
Department of Molecular Genetics, VIB
Laboratory of Neurogenetics, Institute Born-Bunge
University of Antwerp
BSc Psychology, University of Liège, Belgium, 1996
MSc Psychology, University of Liège, Belgium, 1999
PhD Biomedical Science, University of Liège, Belgium, 2007
Postdoctoral research, McGill University, Montreal, Canada,
2007-2010
Current Position
Postdoctoral scientist at VIB Department of Molecular Genetics
E-mail: delphine.bouhy@molgen.vib-ua.be
Phone: +32 3 265 1026
Current Project Members
PhD Student: Thomas Geuens, MSc
Laboratory Technician: Vicky De Winter, BSc
Keywords
Transgenic mouse models – Inherited peripheral neuropathies – small heat shock
protein (sHSP)
Science
My interest in the nervous system has been driven by a top-down approach, from a
behavioural to a molecular point of view. As I find exiting to unravel the complexity
of a system by dissecting its failures, I naturally plunged into the study of axonal
degeneration. After spending 7 years trying to improve axonal regeneration after
spinal cord injury, I joined the Peripheral Neuropathy group of the department of
Molecular Genetics to dissect the pathomechanisms underlying peripheral
neuropathies.
My project aims at understanding how mutations in small heat shock proteins HSPB1
and HSPB8 cause axonal Charcot-Marie-Tooth (CMT2) neuropathy and distal
hereditary motor neuropathy (distal HMN). These inherited diseases are caused by a
length-dependent degeneration of peripheral nerves resulting in severe weakness
and wasting of distal muscle in the lower limbs. Our lab identified mutations in the
small heat-shock proteins 22 (HSPB8) and 27 (HSPB1) to be associated with distal
HMN and CMT2. Both HSPB1 and HSPB8 are widely expressed genes coding for
chaperone proteins with essential cellular functions. The first hypothesis was that the
neuropathy would result from inefficient chaperone activity of the mutated forms of
HSPB1 and HSPB8 in specific regions like the distal neurites and synaptic terminals.
However, we recently showed that in contrast to other chaperonopathies some
mutations in HSPB1 increase their chaperone activity and their binding properties.
My project is to further understand how the mutations in HSPB1 and HSPB8 lead to
axonopathy by using transgenic mouse lines. We generated transgenic mice that
express the wild type or selected mutant forms of HSPB1 and HSPB8 using
conditional and knock-in strategies in order to study neurons susceptibility to sHSP
mutation as well as non-cell autonomous components of sHSP mutations in CMT

neuropathies. We will study the phenotype of these transgenic lines and will also use
them to validate our recent results and develop therapeutic strategies.
Recent Grants and Fellowships
Association Belge contre Les Maladies neuro-Musculaires (ABMM) – Research Grant
Period: 2011-2012
Title: ‘Transgenic models to identify Charcot-Marie-Tooth pathomechanisms caused
by mutations in HSPB1 and HSPB8’
Selected Publications
Bouhy D, Ghasemlou N, Lively S, Redensek A, Rathore KI, Schlichter LC, David S:
Inhibition of the Ca²+-dependent K+ channel, KCNN4/KCa3.1, improves tissue
protection and locomotor recovery after spinal cord injury. Journal of Neuroscience.
Epub 2011 (I.F.: 7.115) (PMID: 22072681)
Ghasemlou N, Bouhy D, Yang J, Lopez-Vales R, Haber M, Thuraisingam T, He G,
Radzioch D, Ding, David S : Beneficial effects of secretory leukocyte protease
inhibitor after spinal cord injury. Brain. Epub 2010 (I.F.: 9.230) (PMID: 20047904)
Girolami E, Bouhy D, Haber M, Johnson H, David S: Differential expression and
potential role of SOCS1 and SOCS3 in Wallerian degeneration in injured peripheral
nerve. Exp Neurol. Epub 2009 (I.F.: 4.436) (PMID: 19576891)
Bouhy D, Malgrange B, Multon S, Poirrier A-L, Scholtes F, Schoenen J and Franzen R
(2006) Delayed GM-CSF treatment stimulates axonal regeneration and functional
recovery in paraplegic rats via an increased BDNF expression by endogenous
macrophages. The FASEB Journal. Epub 2006 (I.F.: 7.049) (PMID: 16636109)
All Publications