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catalase
converts
excess
H2O2
to
H2O
2H2O2
->
2H2O
+
O2
BREAKDOWN
OF
FATTY
ACID
MOLECULES,
oxidation
shortens
the
alkyl
chains
of
fatty
acids
sequentially
in
blocks
of
two
carbon
atoms
at
a
time,
thereby
converting
the
fatty
acids
to
acetyl
CoA
PLASMALOGENS,
most
abundant
class
of
phospholipids
in
MYELIN
(12-31)
peroxisomal
disorders
lead
to
neurological
disease,
Plasmalogen
deficiencies
Zellweger
syndrome,
in
which
a
defect
in
importing
proteins
->
peroxisomal
deficiency
Severe
abnormalities
in
their
brain,
liver,
and
kidneys,
and
they
die
soon
after
birth
mutation
in
the
gene
encoding
peroxin
Pex2
defective
receptor
for
the
N-
terminal
import
signal
SS:
three
amino
acids
(SerLysLeu)
located
at
the
C-terminus,
import
signal
Soluble
receptor
proteins
in
the
cytosol,
which
recognize
the
targeting
signals,
and
docking
proteins
on
the
cytosolic
surface
(peroxins)
Import
resemble
protein
transport
into
the
nucleus
(folded
and
oligomeric
prots)
ER
>
than
half
of
the
total
membrane
ER
lumen,
more
than
10%
of
the
total
cell
volume
lipid
and
protein
biosynthesis
cells
organelles,
ER,
Golgi
apparatus,
lysosomes,
endosomes,
secretory
vesicles,
plasma
membrane
lipids
for
mitochondrial
and
peroxisomal
membranes
proteins
secreted
@
exteriorlumen
of
the
ER,
Golgi
apparatus,
or
lysosomes
Protien
import
is
a
co-translational
Ribosome
attached
directly
to
the
ER
membrane
ROUGH
ER
SMOOTH
ER
HEPATOCYTE,
also
has
abundant
smooth
ER.
principal
site
of
production
of
lipoprotein
particles,
which
carry
lipids
via
the
bloodstream,
enzymes
synthesize
lipid
components
of
the
particles
detoxification
reactions
are
carried
out
by
the
cytochrome
P450
enzymes
INTRACELLULAR
CA2+
STORE
Ca2+
pump
transports
Ca2+
from
the
cytosol
into
the
ER
lumen
High
concentration
of
Ca2+-binding
proteins
MICROSOMES
small
authentic
versions
of
the
ER,
protein
translocation,
protein
glycosylation,
Ca2+
uptake
and
release,
and
lipid
synthesis
(12-37)
ER
captures
selected
proteins
from
the
cytosol
(12-38)
transmembrane
proteins
water-soluble
proteins
directed
to
the
ER
membrane
by
an
ER
signal
sequence
N-terminal
leader
peptide,
signal
peptidase
signal-recognition
particle
(SRP),
SRP
receptor
halts
protein
synthesis
when
signal
peptide
has
emerged
from
the
ribosome
Cysteines
exposed
to
either
the
extracellular
space
or
the
lumen
in
the
secretory
and
endocytic
pathways
are
disulfide-bonded
ER
resident
protein
is
the
chaperone
protein
BiP
BiP
recognizes
incorrectly
folded
proteins
(Hsp70
family),
ATPase
Covalent
addition
of
sugars
to
proteins,
glycoproteins
(N-linked)
->
Golgi
apparatus,
lysosomes,
plasma
membrane,
or
extracellular
space
ER
chaperone
proteins,
calnexin
and
calreticulin
require
Ca2+
(LECTINS)
Sugars
help
achieve
properly
folded
or
oligomeric
state.
Misfolded
proteins
are
exported
from
the
ER
back
into
the
cytosol,
degraded
ER
enzymes,
attach
a
glycosylphosphatidyl-inositol
(GPI)
anchor
to
the
C-
terminus
of
some
membrane
proteins
destined
for
the
plasma
membrane
glycosylation
all
of
the
major
classes
of
lipids,
phospholipids
and
cholesterol,
required
for
the
production
of
new
cell
membranes
Mbr
synthesis