PMID- 29315582
OWN - NLM
STAT- MEDLINE
DCOM- 20190508
LR  - 20190508
IS  - 1471-4159 (Electronic)
IS  - 0022-3042 (Print)
IS  - 0022-3042 (Linking)
VI  - 145
IP  - 3
DP  - 2018 May
TI  - A dual role for Integrin alpha6beta4 in modulating hereditary neuropathy with liability 
      to pressure palsies.
PG  - 245-257
LID - 10.1111/jnc.14295 [doi]
AB  - Peripheral myelin protein 22 (PMP22) is a component of compact myelin in the 
      peripheral nervous system. The amount of PMP22 in myelin is tightly regulated, 
      and PMP22 over or under-expression cause Charcot-Marie-Tooth 1A (CMT1A) and 
      Hereditary Neuropathy with Pressure Palsies (HNPP). Despite the importance of 
      PMP22, its function remains largely unknown. It was reported that PMP22 interacts 
      with the beta4 subunit of the laminin receptor alpha6beta4 integrin, suggesting that alpha6beta4 
      integrin and laminins may contribute to the pathogenesis of CMT1A or HNPP. Here 
      we asked if the lack of alpha6beta4 integrin in Schwann cells influences myelin 
      stability in the HNPP mouse model. Our data indicate that PMP22 and beta4 integrin 
      may not interact directly in myelinating Schwann cells, however, ablating beta4 
      integrin delays the formation of tomacula, a characteristic feature of HNPP. In 
      contrast, ablation of integrin beta4 worsens nerve conduction velocities and 
      non-compact myelin organization in HNPP animals. This study demonstrates that 
      indirect interactions between an extracellular matrix receptor and a myelin 
      protein influence the stability and function of myelinated fibers.
CI  - (c) 2018 International Society for Neurochemistry.
FAU - Poitelon, Yannick
AU  - Poitelon Y
AUID- ORCID: 0000-0001-9868-1569
AD  - Hunter James Kelly Research Institute, University at Buffalo, Buffalo, New York, 
      USA.
AD  - Department of Biochemistry, University at Buffalo, Buffalo, New York, USA.
FAU - Matafora, Vittoria
AU  - Matafora V
AUID- ORCID: 0000-0002-4311-998X
AD  - Division of Genetics and Cell Biology, San Raffaele Hospital, Milan, Italy.
FAU - Silvestri, Nicholas
AU  - Silvestri N
AD  - Department of Neurology, University at Buffalo, Buffalo, New York, USA.
FAU - Zambroni, Desiree
AU  - Zambroni D
AUID- ORCID: 0000-0003-4027-1094
AD  - Division of Genetics and Cell Biology, San Raffaele Hospital, Milan, Italy.
FAU - McGarry, Claire
AU  - McGarry C
AD  - Department of Biochemistry, University at Buffalo, Buffalo, New York, USA.
FAU - Serghany, Nora
AU  - Serghany N
AD  - Department of Biochemistry, University at Buffalo, Buffalo, New York, USA.
FAU - Rush, Thomas
AU  - Rush T
AD  - Department of Biochemistry, University at Buffalo, Buffalo, New York, USA.
FAU - Vizzuso, Domenica
AU  - Vizzuso D
AD  - Hunter James Kelly Research Institute, University at Buffalo, Buffalo, New York, 
      USA.
AD  - Division of Genetics and Cell Biology, San Raffaele Hospital, Milan, Italy.
FAU - Court, Felipe A
AU  - Court FA
AUID- ORCID: 0000-0002-1384-2552
AD  - Division of Genetics and Cell Biology, San Raffaele Hospital, Milan, Italy.
AD  - Center for Integrative Biology, Universidad Mayor de Chile, Santiago, Chile.
FAU - Bachi, Angela
AU  - Bachi A
AUID- ORCID: 0000-0003-4842-6556
AD  - Division of Genetics and Cell Biology, San Raffaele Hospital, Milan, Italy.
FAU - Wrabetz, Lawrence
AU  - Wrabetz L
AUID- ORCID: 0000-0003-3360-1032
AD  - Hunter James Kelly Research Institute, University at Buffalo, Buffalo, New York, 
      USA.
AD  - Department of Biochemistry, University at Buffalo, Buffalo, New York, USA.
AD  - Division of Genetics and Cell Biology, San Raffaele Hospital, Milan, Italy.
AD  - Department of Neurology, University at Buffalo, Buffalo, New York, USA.
FAU - Feltri, Maria Laura
AU  - Feltri ML
AUID- ORCID: 0000-0002-2276-9182
AD  - Hunter James Kelly Research Institute, University at Buffalo, Buffalo, New York, 
      USA.
AD  - Department of Biochemistry, University at Buffalo, Buffalo, New York, USA.
AD  - Division of Genetics and Cell Biology, San Raffaele Hospital, Milan, Italy.
AD  - Department of Neurology, University at Buffalo, Buffalo, New York, USA.
LA  - eng
GR  - R01 NS045630/NS/NINDS NIH HHS/United States
GR  - R01 NS100464/NS/NINDS NIH HHS/United States
GR  - R56 NS096104/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20180213
PL  - England
TA  - J Neurochem
JT  - Journal of neurochemistry
JID - 2985190R
RN  - 0 (Integrin alpha6beta4)
RN  - 0 (Myelin Proteins)
RN  - 0 (Pmp22 protein, mouse)
RN  - Tomaculous neuropathy
SB  - IM
MH  - Animals
MH  - Arthrogryposis/*metabolism/pathology
MH  - Hereditary Sensory and Motor Neuropathy/*metabolism/pathology
MH  - Integrin alpha6beta4/*metabolism
MH  - Mice
MH  - Mice, Knockout
MH  - Myelin Proteins/metabolism
MH  - Myelin Sheath/metabolism/pathology
MH  - Schwann Cells/*metabolism/pathology
PMC - PMC5924464
MID - NIHMS933345
OTO - NOTNLM
OT  - HNPP
OT  - Integrin beta4
OT  - Pmp22
OT  - Schwann cells
OT  - myelin
OT  - peripheral nerve
EDAT- 2018/01/10 06:00
MHDA- 2019/05/09 06:00
PMCR- 2019/05/01
CRDT- 2018/01/10 06:00
PHST- 2017/07/25 00:00 [received]
PHST- 2017/12/14 00:00 [revised]
PHST- 2017/12/21 00:00 [accepted]
PHST- 2018/01/10 06:00 [pubmed]
PHST- 2019/05/09 06:00 [medline]
PHST- 2018/01/10 06:00 [entrez]
PHST- 2019/05/01 00:00 [pmc-release]
AID - 10.1111/jnc.14295 [doi]
PST - ppublish
SO  - J Neurochem. 2018 May;145(3):245-257. doi: 10.1111/jnc.14295. Epub 2018 Feb 13.