Fibrodysplasia Ossificans Progressiva | ACVR1(c.617G>A; R206H) = Klassisk FOP
Dr Bob Pignolo i mitten tillsammans med hela forskar-teamet

21a FOP-forskningsrapporten – 2012

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Nyss hemkommen från 4dagars arbetsledarutbildning i Rom hittar jag spännande läsning i min e-mail. Den senaste forskningsrapporten 34sidor intressant läsning, men jag börjar med att lägga ut den här. Nedan kan du läsa innehållsförteckningen och även ladda ner hela pdf filen.

Frederick S. Kaplan, MD
Robert J. Pignolo, MD, PhD
Eileen M. Shore, PhD

Maj 2012

Innehåll;
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Part I – The Architects and the Builders. . . . . . . . . . . . . . . . . . .9
FOP: A Blueprint for the Metamorphosis . . . . . . . . . . . . . . . . . . . . . . . . . .9
The Hidden Architect of Skeletal Metamorphosis in FOP . . . . . . . . . . . . 9
Inflammation and FOP: Triggering the Metamorphosis . . . . . . . . . . . . .10
The Microenvironment of the Metamorphosis. . . . . . . . . . . . . . . . . . . . . 10
Stem Cells and FOP: The Master Builders of the Metamorphosis. . . . . 10
Emerging Principles of Skeletal Metamorphosis in FOP . . . . . . . . . . . . . 11
Treatment Strategies for Inhibiting Skeletal Metamorphosis in FOP . .11

Part II – Stopping the Metamorphosis. . . . . . . . . . . . . . . . . . . . 12
Workshop for a Cure. . . . . . . . . . . . . . . . . . ..  . . . . . . . . . . . . . . . . . . . . . . . .12
Comprehensive Survey of FOP Flare-ups: Essential Background
for the Design of a Clinical Trial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .17
A Mouse with FOP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .18
Architects of Sabotage: Backing-up the Runaway Train and
Blowing-up the Bridge. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Blocking Activity of the FOP Receptor (ACVR1/ALK2). . . . . . . . . . . . . .20
FOP: From A Fishing Expedition to a Master Plan . . . . . . . . . . . . . . . . . .21
Foremen on the Construction Site: Nerves and Immune System
Trigger the Building of a Second Skeleton. . . . . . . . . . . . . . . . . . . . . . . . . 22
The Architect’s Drawing Board. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25
The Architects of Change & the Sounds of Silence. . . . . . . . . . . . . . . . . . .26
Architecture at the Atomic Level . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
Shaking the Foundations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .28
The Construction Site . .  . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
FOP Treatment Guidelines: Revised and Updated. . . . . . . . . . . . . . . . . 30
FOP: The Written Word – 2011 . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . 30
FOP: The Spoken Word – 2011 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
The FOP Team: Architects and Builders . . . . . . . . . . . . . . . . . . . . . . . . . .31
FOP: Everyone Can Help . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
Many Thanks . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34

“Nature teaches, but what it teaches is often hidden and obscure. It would be well to consider what may be called the hidden teacher, lest, we become too much concerned with the formalities by which we learn. We think we learn from teachers, and we sometimes do. But the teachers are not always to be found in schools or in great laboratories. Some times what we learn depends on our own power of insight.” ~ Lauren Eiseley – Anthropologist The University of Pennsylvania (The Unexpected Universe)

Marie H Fahlberg
Nominerad till David Legas stipendium 2012.

Stöd FOP-drabbade genom bidrag till FOP-forskningen

Bankgiro 5823-7140

 

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