Autophagy,
Год журнала:
2013,
Номер
9(9), С. 1308 - 1320
Опубликована: Сен. 3, 2013
Amyotrophic
lateral
sclerosis
(ALS)
is
a
fatal
motoneuron
disease
with
no
current
effective
treatment.
Accumulation
of
abnormal
protein
inclusions
containing
SOD1,
TARDBP,
FUS,
among
other
proteins,
pathological
hallmark
ALS.
Autophagy
the
major
degradation
pathway
involved
in
clearance
damaged
organelles
and
aggregates.
Although
autophagy
has
been
shown
to
efficiently
degrade
ALS-linked
mutant
cell
culture
models,
several
studies
suggest
that
impairment
may
also
contribute
pathogenesis.
In
this
report,
we
tested
potential
use
trehalose,
disaccharide
induces
MTOR-independent
autophagy,
development
experimental
Administration
trehalose
SOD1
transgenic
mice
significantly
prolonged
life
span
attenuated
progression
signs.
These
effects
were
associated
decreased
accumulation
aggregates
enhanced
survival.
The
protective
increased
levels
motoneurons.
Cell
experiments
demonstrated
led
by
NSC34
cells
protected
primary
motoneurons
against
toxicity
conditioned
media
from
astrocytes.
At
mechanistic
level,
treatment
significant
upregulation
expression
key
autophagy-related
genes
at
mRNA
level
including
Lc3,
Becn1,
Sqstm1
Atg5.
Consistent
these
changes,
administration
nuclear
translocation
FOXO1,
an
important
transcription
factor
activation
neurons.
This
study
suggests
enhancers
for
Autophagy,
Год журнала:
2012,
Номер
8(4), С. 445 - 544
Опубликована: Апрель 1, 2012
In
2008
we
published
the
first
set
of
guidelines
for
standardizing
research
in
autophagy.
Since
then,
on
this
topic
has
continued
to
accelerate,
and
many
new
scientists
have
entered
field.
Our
knowledge
base
relevant
technologies
also
been
expanding.
Accordingly,
it
is
important
update
these
monitoring
autophagy
different
organisms.
Various
reviews
described
range
assays
that
used
purpose.
Nevertheless,
there
continues
be
confusion
regarding
acceptable
methods
measure
autophagy,
especially
multicellular
eukaryotes.
A
key
point
needs
emphasized
a
difference
between
measurements
monitor
numbers
or
volume
autophagic
elements
(e.g.,
autophagosomes
autolysosomes)
at
any
stage
process
vs.
those
flux
through
pathway
(i.e.,
complete
process);
thus,
block
macroautophagy
results
autophagosome
accumulation
differentiated
from
stimuli
result
increased
activity,
defined
as
induction
coupled
with
delivery
to,
degradation
within,
lysosomes
(in
most
higher
eukaryotes
some
protists
such
Dictyostelium)
vacuole
plants
fungi).
other
words,
investigators
field
understand
appearance
more
does
not
necessarily
equate
fact,
cases,
accumulate
because
trafficking
without
concomitant
change
biogenesis,
whereas
an
increase
autolysosomes
may
reflect
reduction
degradative
activity.
Here,
present
selection
interpretation
use
by
who
aim
examine
related
processes,
well
reviewers
need
provide
realistic
reasonable
critiques
papers
are
focused
processes.
These
meant
formulaic
rules,
appropriate
depend
part
question
being
asked
system
used.
addition,
emphasize
no
individual
assay
guaranteed
one
every
situation,
strongly
recommend
multiple
guidelines,
consider
various
assessing
what
information
can,
cannot,
obtained
them.
Finally,
discussing
merits
limits
particular
assays,
hope
encourage
technical
innovation
New England Journal of Medicine,
Год журнала:
2017,
Номер
377(2), С. 162 - 172
Опубликована: Июль 12, 2017
Progress
has
been
made
in
understanding
the
genetic
defects
and
pathophysiology
of
this
crippling
motor
neuron
disease
(commonly
called
Lou
Gehrig's
disease).
However,
information
not
yet
led
to
a
successful
intervention
that
alters
course
disease.
