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Progerin (UniProt# P02545-6) is a
truncated version of the
lamin A
protein involved in the
pathology of Hutchinson–Gilford
progeria syndrome. Progerin...
- together. When this gene mutates, an
abnormal form of
lamin A
protein called progerin is produced.
Progeroid syndromes are a
group of
diseases that
cause individuals...
- is an oral
medication that
helps prevent the
buildup of
defective progerin or
progerin-like protein. The
effectiveness of
lonafarnib for the
treatment of...
- LMNA mRNA,
therefore producing abnormal lamin A protein, also
known as
progerin. The
mutations activate a
cryptic splice site
within exon 11 of the gene...
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alterations in
alternative RNA
splicing that
produce senescent toxins such as
progerin,
which degrades tissue and
makes it more
prone to failure. BRAFV600E and...
- Consequently, no
mature lamin A is formed, and a
farnesylated mutant prelamin A (
progerin) ac****ulates in cells. The
nuclear lamina consist of a two-dimensional...
- and C; the
mutation causes the
production of
truncated lamins A,
called progerins,
which cause the
premature ageing typical of the disease. In 2006, the...
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being deleted. This
results in a
truncated lamin A
precursor (a.k.a.
progerin or LaminAΔ50).
After being translated, a
farnesol is
added to prelamin...
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Adrienne D.; Collins,
Francis S. (2005-09-06). "Inhibiting
farnesylation of
progerin prevents the
characteristic nuclear blebbing of Hutchinson-Gilford progeria...
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Administration approved lonafarnib,
which helps prevent buildup of
defective progerin and
similar proteins. A
clinical trial in 2018
pointed to significantly...
