NUCLEAR PORES

These pores cross both of the membranes, providing a channel that allos free
movement of small molec!les and ions"
The movement of larger molec!les s!ch as proteins is caref!ll# controlled, and
re$!ires active transport reg!lated b# carrier proteins" N!clear transport is cr!cial
to cell f!nction, as movement thro!gh the pores is re$!ired for both gene
e%pression and chromosomomal maintenance"
R&'OSO(E
Ribosome are fo!nd in the c#toplasm and on the endoplasmic retic!l!m" Their
job is to make proteins needed in the cell for everyday life. The n!cle!s
tells the ribosomes hich protein to ma)e, so that the cell can or) e*cientl#"
The n!cle!s also ma)es the ribosomes and sends them o!t for or)"
The ribosome f!nction in the e%pression of the genetic code from n!cleic acid into
protein, in a process called translation" Ribosomes do this b# catal#+ing the
assembl# of individ!al amino acids into pol#peptide chains, this involves binding a
messenger RNA and then !sing this as a template to -oin together the correct
se$!ence of amino acids" This reaction !ses adapters called transfer RNA
molec!les, hich read the se$!ence of the mesenger RNA and are attached to the
amino acids"
L.SOSO(ES
The name l#sosome derives from the gree) ords l#sis, hich means dissol!tion
or destr!ction, and soma, hich means bod#
L#sosomes are organelles containing digestive en+#mes /acid h#drolases0" The#
are fo!nd in animal cells, hile in plant cells the same roles are performed b# the
vac!ole"
The# digest e%cess or orn1o!t organelles, food particles, and eng!lfed vir!ses or
bacteria"
The si+e of l#sosomes varies from 2"312"4 mm" At p5 6"7, the interior of the
l#sosomes is acidic compared to the slightl# al)aline c#tosol /p5 8"40"
L.SOSO(AL STORA9E :&SOR:ERS /LS:0
The lsosomal storage diseases have a broad spectr!m of clinical phenot#pes" &n
addition the age of onset, severit# of s#mmptoms and central nervo!s s#stem
manifestation can var# mar)edl# ithin a single disorder t#pe" Several l#sosomal
storage disorders s!ch as for e%ample 9a!cher disesase, Ta# Sachs disease,
Pompe disease, beta1galactosidase de;cienc# have infantile, -!venile and ad!lt
forms"
En+#me replacement therap#
&mportant from a therape!tic point of vie is that l#sosomal proteins added to the
e%tracell!lar space ill reach the l#sosomes, !s!all# via lectins on the plasma
membrane as the mannose <1phosphate receptor and the mannose receptor"
PERO=&(ES
Peroximes are organelles from the microbod# famil# and are present in almost
all e!)ar#otic cells"
The# participate in the metabolism of fatt# acids and man# other metabolites"
Pero%isomes harbor en+#mes that rid the cell of to%ic pero%ides"
Pero%isomes are bo!nd b# a single membrane that separates their contents from
the c#tosol and contain membrane proteins critical for vario!s f!nction, s!ch as
importing proteins into the organelles and aiding in proliferation"
Pero%isomes can replicate b# enlarging anf then dividing
Pero%isomes ere identi;ed as organelles b# the belgian c#tologist Christian de
:!ve in 3><8"
C.TOS?ELETON
The c#tos)eleton is a netor) of ;bers thro!gho!t the cell@s c#toplasm that helps
the cell maintain its shape and gives s!pport to the cell" A variet# of cell!lar
organelles are held in place b# the c#tos)eleton"
establishing cell shape
providing mechanical strength
locomotion
chromosome separation in mitosis and meiosis
intracell!lar transport of organelles
(&TOC5ON:R&A
The# are organelles that act li)e a digestive system that ta)es in n!trients,
brea)s them don, and creates energ# for the cell" The process of creating cell
energ# is )non as cellular respiration"
(itochondria are ver# small organelles" .o! might ;nd cells ith several tho!sand
mitochondria" The n!mber depens on hat the cell needs to do" &f the p!pose of
the cell is to transmit nerve imp!lses, there ill be feer mitochondria than in a
m!scle cell that needs loads of energ#" &f the cell feels it is not getting eno!gh
energ# to s!rvive, more mitochondria can be created" Sometimes the# can even
gro, move, and combine ith other mitochondria, depending on the cell@s needs"
CELL '&OLO9.
The st!d# of the activities, f!nction, properties and str!ct!re of cells"
Cell is the f!ndamental !nit, ho!sing the genetic material and biochemical
organi+ation hich acco!nt for the e%istence of life"
All cells store information in genes made of :NA
The genetic code !sed in the genes is the same in all species of cells
All cells decode the genes in their :NA b# a RNA s#stem that translates genetic
informationinto proteins
All cells s#nthesi+e proteins b# !sing the ribosome
Proteins govern the activities, f!nction, and str!ct!res in all cells
All cells need energ# to operate A ATP
All cells are enclosed b# a plasma membrane composed of lipid and protein
molec!les"
(OLECULAR '&OLO9.
To st!d# str!ct!ral and f!nctional properties of biological s#stems, p!rs!ed ithin
the conte%t of !nderstanding the roles of the various molecules in living cells
and the relationship beteen them"
To st!d# the formation, str!ct!re and f!nction of macromolec!les /n!cleic acids
and proteins0, their role in cell replication and transmission of genetic information"
:eveloping the methods to st!d# :NA se$!ence and :NA m!tation
STRUCTURE '&OLO9.
The combination of molec!lar biolog#, biochemistr# and bioph#sics, to st!d# the
molec!lar str!ct!re of biological macromolec!les, proteins and n!cleic acids, ho
the# ac$!ire the str!ct!re aBect their f!nction"
(ethodsC
Cr#stallograph#, N(R, Ultra fast laser spectroscop# electron microscop#, :!al
polari+ation &nterferometr#, circ!lar diachronic"
&NTRACELLULAR OR9ANELLES
N!cle!s, N!cleol!s, Cromatin, Ribosome
(embrane A n!clear and plasma membrane
C#tosol or c#toplasm
Endoplasmic retic!l!m
9olgi apparat!s and secretar# gran!les
(itochondria
L#sosomes
Pero%isomes
(icrot!b!les and micro;laments
Centriole
NU?L&R pori1pori
Pori1pori ini lintas )ed!a membran, men#edia)an sal!ran #ang mem!ng)in)an
pergera)an bebas mole)!l )ecil dan ion"
9era)an mole)!l besar seperti protein di)ontrol dengan hati1hati, dan
memb!t!h)an transpor a)tif diat!r oleh protein carrier" transportasi n!)lir sangat
penting !nt!) f!ngsi sel, sebagai gera)an melal!i pori1pori diperl!)an !nt!)
)ed!a e)spresi gen dan pemeliharaan chromosomomal"
Ribosom
Ribosom ditem!)an di sitoplasma dan di reti)!l!m endoplasma" T!gas mere)a
adalah !nt!) memb!at protein dib!t!h)an dalam sel !nt!) )ehid!pan sehari1hari"
inti ini mencerita)an protein ribosom #ang memb!at, sehingga sel ini dapat
be)er-a dengan e;sien" inti ini -!ga memb!at ribosom dan mengirim mere)a
)el!ar !nt!) be)er-a"
D!ngsi ribosom dalam e)spresi )ode geneti) dari asam n!)leat men-adi protein,
dalam proses #ang diseb!t ter-emahan" Ribosom mela)!)an hal ini dengan )atalis
ma-elis asam amino )e rantai polipeptida individ!, ini melibat)an mengi)at s!at!
messenger RNA dan )em!dian mengg!na)an ini sebagai template !nt!)
bergab!ng bersama1sama dengan !r!tan #ang benar dari asam amino" Rea)si ini
mengg!na)an adapter diseb!t transfer mole)!l RNA, #ang membaca !r!tan
mesenger RNA dan #ang mele)at pada asam amino"
Lisosom
Nama lisosom berasal dari )ata .!nani lisis, #ang berarti pemb!baran ata!
pengr!sa)an, dan soma, #ang berarti baha t!b!h
Lisosom adalah organel #ang mengand!ng en+im pencernaan /h#drolases asam0"
(ere)a ditem!)an di sel hean, sedang)an pada sel t!mb!han peran #ang sama
dila)!)an oleh va)!ola"
(ere)a mencerna organel )elebihan ata! !sang, parti)el ma)anan, dan menelan
vir!s ata! ba)teri"
U)!ran lisosom bervariasi 2,312,4 mm" Pada p5 6,7, interior dari lisosom adalah
asam dibanding)an dengan sitosol sedi)it basa /p5 8"40"
PEN.&(PANAN L.SOSO(AL 9AN99UAN /LS:0
Pen#a)it pen#impanan lsosomal memili)i spe)tr!m #ang l!as dari fenotipe )linis"
Selain !sia onset, )eparahan s#mmptoms dan manifestasi sistem saraf p!sat
dapat bervariasi n#ata dalam -enis gangg!an t!nggal" 'eberapa gangg!an
pen#impanan l#sosomal seperti misaln#a disesase 9a!cher, pen#a)it Ta# Sachs,
pen#a)it Pompe, de;siensi beta1gala)tosidase telah )e)ana)1)ana)an, bent!)
rema-a dan deasa"
Terapi penggantian en+im
Penting dari s!d!t pandang terape!ti) adalah baha protein l#sosomal
ditambah)an )e r!ang e)strasel!ler a)an mencapai lisosom, biasan#a melal!i
lectins pada membran plasma reseptor <1fosfat mannose dan reseptor mannose"
PERO=&(ES
Pero%imes adalah organel dari )el!arga microbod# dan hadir di hampir sem!a sel
e!)arioti)"
(ere)a berpartisipasi dalam metabolisme asam lema) dan metabolit lainn#a"
Pero)sisom pelab!han en+im #ang menghilang)an sel dari pero)sida berac!n"
Pero)sisom teri)at oleh membran t!nggal #ang memisah)an isin#a dari sitosol
dan mengand!ng protein membran penting !nt!) berbagai f!ngsi, seperti
mengimpor protein )e dalam organel dan membant! dalam proliferasi"
Pero)sisom bisa menir! dengan memperbesar AND )em!dian membagi
Pero)sisom diidenti;)asi sebagai organel oleh c#tologist Christian de :!ve 'elgia
pada tah!n 3><8"
Sitos)eleton
sitos)eleton adalah -aringan serat di sel!r!h sitoplasma sel sel #ang membant!
men-aga bent!) dan memberi d!)!ngan )epada sel" 'erbagai organel sel!ler
ditahan oleh sitos)eleton terseb!t"
menetap)an bent!) sel
memberi)an )e)!atan me)ani)
da#a penggera)
pemisahan )romosom dalam mitosis dan meiosis
intrasel!ler organel transport
(ito)ondria
(ere)a adalah organel #ang bertinda) seperti sistem pencernaan #ang
mengambil di gi+i, istirahat mere)a, dan mencipta)an energi !nt!) sel" Proses
mencipta)an energi sel di)enal sebagai respirasi sel"
(ito)ondria adalah organel sangat )ecil" Anda m!ng)in menem!)an beberapa
rib! sel1sel dengan mito)ondria" Nomor depens pada sel apa #ang har!s
dila)!)an" Ei)a sel ?eg!naann#a adalah !nt!) mengirim)an imp!ls saraf, a)an
ada lebih sedi)it daripada di mito)ondria sel otot #ang memb!t!h)an ban#a)
energi" Ei)a sel merasa tida) mendapat)an energi #ang c!)!p !nt!) bertahan
hid!p, mito)ondria lebih dapat dib!at" ?adang1)adang mere)a bah)an bisa
t!mb!h, bergera), dan menggab!ng)an dengan mito)ondria #ang lain,
tergant!ng pada )eb!t!han sel"
SEL '&OLO9&
St!di tentang )egiatan, f!ngsi, sifat dan str!)t!r sel"
Sel adalah !nit dasar, per!mahan bahan geneti) dan bio)imia organisasi #ang
men-elas)an )eberadaan )ehid!pan"
Sem!a sel men#impan informasi dalam gen #ang terb!at dari :NA
?ode geneti) #ang dig!na)an dalam gen adalah sama di sem!a -enis sel
Sem!a sel1decode gen dalam :NA mere)a dengan sistem RNA #ang
mener-emah)an protein informationinto geneti)
Sem!a sel mensintesis protein dengan mengg!na)an ribosom
Protein mengat!r )egiatan, f!ngsi, dan str!)t!r di sel!r!h sel
Sem!a sel memb!t!h)an energi !nt!) beroperasi 1 ATP
Sem!a sel #ang tert!t!p oleh membran plasma terdiri dari lipid dan mole)!l
protein"
'&OLO9& (OLE?UL
Unt!) mempela-ari sifat str!)t!ral dan f!ngsional dari sistem biologi, di)e-ar
dalam )onte)s pemahaman peranan dari berbagai mole)!l dalam sel hid!p dan
h!b!ngan antara mere)a"
Unt!) mempela-ari pembent!)an, str!)t!r dan f!ngsi ma)romole)!l /asam
n!)leat dan protein0, peran mere)a dalam repli)asi sel dan transmisi informasi
geneti)"
(engembang)an metode !nt!) mempela-ari !r!tan :NA dan m!tasi :NA
STRU?TUR '&OLO9&
?ombinasi biologi mole)!lar, bio)imia dan bio;si)a, !nt!) mempela-ari str!)t!r
mole)!l ma)romole)!l biologis, protein dan asam n!)leat, bagaimana mere)a
memperoleh str!)t!r mempengar!hi f!ngsi mere)a"
(etodeC
?ristalogra;, N(R, !ltra cepat spe)tros)opi laser mi)ros)op ele)tron, :!al
polarisasi interferometri, dia)ronis meling)ar"
&ntrasel!ler organel
&nti, N!)leol!s, Cromatin, ribosom
(embran 1 membran n!)lir dan plasma
Sitosol ata! sitoplasma
Reti)!l!m endoplasma
Aparat 9olgi dan b!tiran se)retaris
(ito)ondria
Lisosom
Pero)sisom
(i)rot!b!l!s dan mi)ro
Sentriol