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Profibrillin 1

Known as: Profibrillin-1 
National Institutes of Health

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fibrillin-1

Papers overview

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2003
2003
Profibrillin‐1 maturation by human dermal fibroblasts: Proteolytic processing and molecular chaperones
Fibrillin‐1 is synthesized as a proprotein that undergoes proteolytic processing in the unique C‐terminal domain by a member of… 
Highly Cited
1998
Highly Cited
1998
Evidence for furin-type activity-mediated C-terminal processing of profibrillin-1 and interference in the processing by certain mutations.
Fibrillin-1 is a major component of the 10 nm microfibrils of the extracellular matrix (ECM). It is synthesized as an… 
1995
1995
Truncated profibrillin of a Marfan patient is of apparent similar size as fibrillin: intracellular retention leads to over-N-glycosylation.
We studied profibrillin-1 (proFib) synthesis and microfibril formation in cultured fibroblasts from an individual with severe… 
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