HNPP
A Study of
Provocation and Treatment of HNPP
2008 Seed
Grant
Raymond P.
Roos, M.D.
University
of Chicago
Charcot-Marie
Tooth (CMT) comprises a group of inherited peripheral neuropathies. One form of
CMT called hereditary liability to pressure palsies (HNPP) is manifest by
recurrent episodes of traumatic or compressive peripheral neuropathies. HNPP is caused by a deletion of a myelin gene
called PMP-22. It is known that vitamin
C decreases the expression of PMP-22 and progesterone increases its
expression. The thrust of this
application is to investigate whether vitamin C ingestion can influence (and
worsen) HNPP clinical disease and whether progesterone can be used as a
treatment of HNPP. The study will test
the effect of vitamin C and progesterone treatment on the clinical and
pathological disease of an HNPP mouse.
There is
presently no treatment of any form of CMT.
The findings of this proposal may have important implications regarding
our understanding and treatment of HNPP.
As a result of this study, HNPP patients may be cautioned about excess
vitamin C ingestion. In addition, the findings of the present study could lead
to a clinical trial with progesterone in HNPP patients.
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at: http://www.thebrf.org/Research+Topics/HNPP#sthash.PVVpsBDK.dpuf
HNPP
A Study of Provocation and Treatment of HNPP
2008 Seed Grant
Raymond P. Roos, M.D.
University of Chicago
Charcot-Marie Tooth (CMT) comprises a group of
inherited peripheral neuropathies. One form of CMT called hereditary
liability to pressure palsies (HNPP) is manifest by recurrent episodes
of traumatic or compressive peripheral neuropathies. HNPP is caused by a
deletion of a myelin gene called PMP-22. It is known that vitamin C
decreases the expression of PMP-22 and progesterone increases its
expression. The thrust of this application is to investigate whether
vitamin C ingestion can influence (and worsen) HNPP clinical disease and
whether progesterone can be used as a treatment of HNPP. The study
will test the effect of vitamin C and progesterone treatment on the
clinical and pathological disease of an HNPP mouse.
There
is presently no treatment of any form of CMT. The findings of this
proposal may have important implications regarding our understanding and
treatment of HNPP. As a result of this study, HNPP patients may be
cautioned about excess vitamin C ingestion. In addition, the findings of
the present study could lead to a clinical trial with progesterone in
HNPP patients.
HNPP
A Study of Provocation and Treatment of HNPP
2008 Seed Grant
Raymond P. Roos, M.D.
University of Chicago
Charcot-Marie Tooth (CMT) comprises a group of
inherited peripheral neuropathies. One form of CMT called hereditary
liability to pressure palsies (HNPP) is manifest by recurrent episodes
of traumatic or compressive peripheral neuropathies. HNPP is caused by a
deletion of a myelin gene called PMP-22. It is known that vitamin C
decreases the expression of PMP-22 and progesterone increases its
expression. The thrust of this application is to investigate whether
vitamin C ingestion can influence (and worsen) HNPP clinical disease and
whether progesterone can be used as a treatment of HNPP. The study
will test the effect of vitamin C and progesterone treatment on the
clinical and pathological disease of an HNPP mouse.
There
is presently no treatment of any form of CMT. The findings of this
proposal may have important implications regarding our understanding and
treatment of HNPP. As a result of this study, HNPP patients may be
cautioned about excess vitamin C ingestion. In addition, the findings of
the present study could lead to a clinical trial with progesterone in
HNPP patients.
HNPP
A Study of Provocation and Treatment of HNPP
2008 Seed Grant
Raymond P. Roos, M.D.
University of Chicago
Charcot-Marie Tooth (CMT) comprises a group of
inherited peripheral neuropathies. One form of CMT called hereditary
liability to pressure palsies (HNPP) is manifest by recurrent episodes
of traumatic or compressive peripheral neuropathies. HNPP is caused by a
deletion of a myelin gene called PMP-22. It is known that vitamin C
decreases the expression of PMP-22 and progesterone increases its
expression. The thrust of this application is to investigate whether
vitamin C ingestion can influence (and worsen) HNPP clinical disease and
whether progesterone can be used as a treatment of HNPP. The study
will test the effect of vitamin C and progesterone treatment on the
clinical and pathological disease of an HNPP mouse.
There
is presently no treatment of any form of CMT. The findings of this
proposal may have important implications regarding our understanding and
treatment of HNPP. As a result of this study, HNPP patients may be
cautioned about excess vitamin C ingestion. In addition, the findings of
the present study could lead to a clinical trial with progesterone in
HNPP patients.
HNPP
A Study of Provocation and Treatment of HNPP
2008 Seed Grant
Raymond P. Roos, M.D.
University of Chicago
Charcot-Marie Tooth (CMT) comprises a group of
inherited peripheral neuropathies. One form of CMT called hereditary
liability to pressure palsies (HNPP) is manifest by recurrent episodes
of traumatic or compressive peripheral neuropathies. HNPP is caused by a
deletion of a myelin gene called PMP-22. It is known that vitamin C
decreases the expression of PMP-22 and progesterone increases its
expression. The thrust of this application is to investigate whether
vitamin C ingestion can influence (and worsen) HNPP clinical disease and
whether progesterone can be used as a treatment of HNPP. The study
will test the effect of vitamin C and progesterone treatment on the
clinical and pathological disease of an HNPP mouse.
There
is presently no treatment of any form of CMT. The findings of this
proposal may have important implications regarding our understanding and
treatment of HNPP. As a result of this study, HNPP patients may be
cautioned about excess vitamin C ingestion. In addition, the findings of
the present study could lead to a clinical trial with progesterone in
HNPP patients.
HNPP
A Study of Provocation and Treatment of HNPP
2008 Seed Grant
Raymond P. Roos, M.D.
University of Chicago
Charcot-Marie Tooth (CMT) comprises a group of
inherited peripheral neuropathies. One form of CMT called hereditary
liability to pressure palsies (HNPP) is manifest by recurrent episodes
of traumatic or compressive peripheral neuropathies. HNPP is caused by a
deletion of a myelin gene called PMP-22. It is known that vitamin C
decreases the expression of PMP-22 and progesterone increases its
expression. The thrust of this application is to investigate whether
vitamin C ingestion can influence (and worsen) HNPP clinical disease and
whether progesterone can be used as a treatment of HNPP. The study
will test the effect of vitamin C and progesterone treatment on the
clinical and pathological disease of an HNPP mouse.
There
is presently no treatment of any form of CMT. The findings of this
proposal may have important implications regarding our understanding and
treatment of HNPP. As a result of this study, HNPP patients may be
cautioned about excess vitamin C ingestion. In addition, the findings of
the present study could lead to a clinical trial with progesterone in
HNPP patients.
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