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BLOOD COMPOSITION
dr. Husnil Kadri, M.Kes
Biochemistry DeartementMedical !aculty O" #ndalas $ni%ersity
Padan&
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BIOSINTESIS HEMOGLOBIN(PORFIRIN)
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Struktur Porfrin
• Porfrin adalah senyawa siklik ygdibentuk oleh 4 cincin pirol.
• Masing-masing cincin dihubungkan
oleh 4 jembatan metenil (-HC!.• "i#at khas porfrin adalah atom
nitrogennya mampu mengikat ion
logam.• Contoh$
- heme pada Hb mengikat %e
- klorofl pada tumbuhan hijau3
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Beberapa Hemoprotein
Protein Fungi
! Hemog"obin mengangkut okigen #i #a"am#ara$
! Miog"obin men%impan okigen #i #a"amotot
! Sitokrom c keter"ibatan pa#a rantaitranpor e"ektron
! Sitokrom
P&' $i#roki"ai enobiotik
! *ata"ae #egra#ai $i#rogenperoki#a
! Tripto+an
piro"ae oki#ai tripto+an4
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Sintei Heme #i Mitokon#ria
• &' sintesis heme terjadi dalam sel
pembentuk eritrosit pada sumsumtulang
• Heme disintesis dari suksinil )o* +glisin.
• Piridoksal #os#at diperlukan untukmengakti#kan glisin.
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Pengaturan Sintei Heme
• ,nim regulator adalah **-sintase.
• Heme bertindak sebagai regulatornegati# (umpan balik negati#! sintesisenim **- sintase.
• /ika heme meningkat0 maka sintesis**-sintase akan menurun.
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Porfria
• Merupakan gangguan genetikbiosintesis heme.
• 1mumnya autosomal dominan0
kecuali porfria eritropoitik kongenital.• 2ejala$
- nyeri abdomen
- gangguan neuropsikiatri- #otosensitiftas kulit
- bila berat prototipe manusia
srigala 8
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,aar Biokimia Porfria
Mutai ,N-
-bnorma"ita en.im pa#a intei $eme
-kumu"ai -L- / PBG atau -kumu"ai
porfrinogenpenurunan $eme #"m e" / #i ku"it / 0aringan
tubu$
1airan tubu$
Tan#a / ge0a"a Oki#ai pontanporfrinogen
neuropikiatrik men0a#i porfrin
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Terapi Porfria
• Hanya simptomatik.
• 3epresor **-sintase$- glukosa
- hematin (bentuk hidroksida dari
heme!- β-karoten untuk #otosensitiftas
- preparat tabir surya
• )ontraindikasi$ - preparat anestesi
- alkohol
- griseo#ulin 5 barbiturat 10
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!un&si $tama Darah
'. (esirasi)
en&an&*utan O+ dan CO+
+. Nutrisi)
en&an&*utan hasil asorsi usus
-. *s*resi)
en&an&*utan sisa metaoli* *e &in/al,
aru0aru, *ulit, 1 usus
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!un&si $tama Darah
2. Keseiman&an asam0asa
3. Keseiman&an air)
antara sir*ulasi darah dan /arin&an
4. Pen&aturan suhu tuuh
5. Pertahanan terhada in"e*si) oleh sel darah utih 1 antiodi
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!un&si $tama Darah
6. Pen&an&*utan hormon 1en&aturan metaolisme
7. Pen&an&*utan metaolit
'8. Koa&ulasi
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Components o# 6hole
7lood
9ithdra: lood
and lace in tue
' + Centri"u&e
Plasma
;33< o" :hole lood=
!ormed
elements
Bu""y coat>
leu*ocyctes and
latelets
;?'< o" :hole lood=
rythrocytes
;23< o" :hole lood=
• Hematocrit
• Males: 47% ± 5%
• Females: 42% ± 5%
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Ph i l Ch t i ti "
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Physical Characteristics o"
Blood• #%era&e %olume o" lood>
– 3@4 L "or males) 2@3 L "or "emales ;Normo%olemia= – Hyo%olemia 0 lo: lood %olume
– Hyer%olemia 0 hi&h lood %olume
• Aiscosity ;thic*ness= 0 2 0 3 ;:here :ater '=
• The H o" lood is [email protected]) 5.2
• Osmolarity -88 mOsm or 8.- Osm
– This %alue re"lects the concentration o" solutes in thelasma
• Salinity 8.63<
–
(e"lects the concentration o" NaCl in the lood• Temerature is -6°C, sli&htly hi&her than normalE ody
temerature
• Blood accounts "or aroimately 6< o" ody :ei&ht
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Components of Blood
–33< lasma
–
23< cells•77< (BCs
•? '< 9BCs and latelets
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Blood Plasma
•
Blood plasma components: – Water = 90-92%
– Proteins = 6-8%
–
Organic ntrients ! glcose" car#o$drates"amino acids
– &lectroltes ! sodim" potassim" calcim"
c$loride" #icar#onate
– 'onprotein nitrogenos s#stances ! lactic acid"rea" creatinine
– (espirator gases ! o)gen and car#on dio)ide
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*ormed &lements
•*ormed elements comprise +,% of#lood
• &rt$roctes" leoctes" and plateletsmae p t$e formed elements – Onl WBCs are complete cells
– (BCs $a.e no nclei or organelles" andplatelets are /st cell fragments
•ost #lood cells do not di.ide #t arerene1ed # cells in #one marro1
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&rt$roctes (BCs3• Biconca.e disc
– *olding increases srface area 40% more srface area3 – Plasma mem#rane contains spectrin
• 5i.e ert$roctes t$eir fle)i#ilit• ncleate" no centrioles" no organelles
–
&nd reslt - no cell di.ision – 'o mitoc$ondria means t$e generate 7P anaerobically
• Pre.ents consmption of O2 #eing transported
• *illed 1it$ $emoglo#in #3 - 9% of cell contents – # fnctions in gas transport
• # O2 #O2 o)$emoglo#in3
• ost nmeros of t$e formed elements
– *emales: +;4!,;2 million cells
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&rt$roctes (BCs3
Figure 17.3
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&rt$rocte *nction
• &rt$roctes are dedicated to respirator gastransport
• emoglo#in re.ersi#l #inds 1it$ o)gen andmost o)gen in t$e #lood is #ond to
$emoglo#in• Composition of $emoglo#in
– protein called glo#in• made p of t1o alp$a and t1o #eta c$ains
– heme molecle• &ac$ $eme grop #ears an atom of iron" 1$ic$ can #ind to
one o)gen molecle
• &ac$ $emoglo#in molecle t$s can transport formolecles of o)gen
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trctre of emoglo#in
Figure 17.4
H l i
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Hemo&loin
• at mol; # de1asa #3 mempnai>
- + ggs $eme- etiap $eme mengandng ? ion *e2
- + s#nit protein glo#in
- etiap s#nit mengiat ? mol; O2
- ? mol; 5lo#in mengiat ? mol; CO2
• #nit rantai terdiri dari 2 α dan 2 β; - α masing-masing = ?+? asam amino
- β masing-masing = ?+6 asam amino
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Hemo&loin
• O)$emoglo#in ! $emoglo#in #ond to o)gen
– O)gen loading taes place in t$e lngs
• @eo)$emoglo#in ! $emoglo#in after o)gen
diffses into tisses redced #3• Car#amino$emoglo#in ! $emoglo#in #ond to
car#on dio)ide
–
Car#on dio)ide loading taes place in t$etisses
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WBC natom and 7pes
• ll WBCs leoctes3 $a.e a ncles and
no $emoglo#in
• 5ranlar or agranlar classification #asedon presence of ctoplasmic granles made
.isi#le # staining –
granloctes are netrop$ils" eosinop$ils or#asop$ils
– agranloctes are monoces or lmp$octes
@iff ti l WBC C t
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@ifferential WBC Cont
• @etection of c$anges in nm#ers of circlating
WBCs percentages of eac$ tpe3 – indicates infection" poisoning" leemia"
c$emot$erap" parasites or allerg reaction
•
'ormal WBC conts – netrop$ils 60-0% p if #acterial infection3
– lmp$octe 20-2,% p if .iral infection3
– monoctes 4 - 8 % p if fngal
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'etrop$ils 5ranlocte3
• Polmorp$onclear eoctes or Pols
• 'clei = 2 to , lo#es connected # t$in
strands – older cells $a.e more lo#es
– ong cells called #and cells #ecase of
$orses$oe s$aped ncles #and3
• *ine" pale lilac practicall in.isi#le granles
• @iameter is ?0-?2 microns
• 60 to 0% of circlating WBCs
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&osinop$ils 5ranlocte3
• 'cles 1it$ 2 or 4 lo#es connected #
a t$in strand
• arge" niform-sied granles stainorange-red 1it$ acidic des – do not o#scre t$e ncles
•@iameter is ?0 to ?2 microns
• 2 to +% of circlating WBCs
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Basop$ils 5ranlocte3
• arge" dar prple" .aria#le-sied
granles stain 1it$ #asic des
– o#scre t$e ncles
• Drreglar" s-s$aped" #ilo#ed nclei
• @iameter is 8 to ?0 microns
• ess t$an ?% of circlating WBCs
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mp$octe granlocte3
• @ar" o.al to rond ncles
• Ctoplasm s #le in color –
amont .aries from rim of #le to normalamont
• mall cells 6 - 9 microns in diameter
•
arge cells ?0 - ?+ microns in diameter – increase in nm#er dring .iral infections
• 20 to 2,% of circlating WBCs
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• 7 cells – Ctoto)ic 7 cells Eiller 7 cells3
• Bind to ctoto)ic cells eg infected # .irs3
• 1ell
• (elease to)ins into ctoplasm
–
elper 7 cells• ost nmeros
• cti.ate B cells" iller 7 cells
• timlate macrop$ages
–ppressor 7 cells• (eglate acti.ities of ot$er cell tpes
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onocte granlocte3
•'cles is idne or $orse-s$oe s$aped
• argest WBC in circlating #lood – does not remain in #lood long #efore migrating to t$e
tisses
– differentiate into macrop$ages• fi)ed grop fond in specific tisses
– al.eolar macrop$ages in lngs
– pffer cells in li.er
•
1andering grop gat$ers at sites of infection• @iameter is ?2 - 20 microns
• Ctoplasm is a foam #le-gra
• 4 to 8% o circlating WBCs
& i ti F P$ t i i WBC
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&migration F P$agoctosis in WBCs
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$NS$( SL$L#( D#L#M (SPON IM$N
?; Galr limfoid ang mem#ent limfosit
dan s#setna
2; Galr mieloid ang mem#ent sel-sel
fagosit mononlear F polimorfonlear
P'3;
P' terdiri dari:
netrofil" eosinofil" #asofil
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•
Platelets are fragments of mega-aroctes
• 7$eir granles contain serotonin" Ca2"enmes" @P" and platelet-deri.edgro1t$ factor P@5*3
• Platelets fnction in t$e clottingmec$anism # forming a temporar
plg t$at $elps seal #reas in #lood.essels
• Platelets not in.ol.ed in clotting areept inacti.e # 'itric O)ide 'O3 and
rosta landins
Platelets
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Protein Plasma
- Bagian tama nsr padat dalam plasma;
- Eonsentrasi total protein plasma -",
g
?; alting-ot 'a2O+ 24%" dll3
2; &letroforesis
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Hone &lectrop$oresis of Plasma
Proteins
- +
pI6.0 5.6 5.1 4.7
globulins albumin
γ β α
α
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Protein Plasma
?; e#agian #esar disintesis di $epar;
2; Immna disintesis s#g preprotein padapoliri#osom teriat mem#ran; Preprotein
aan mengalami modifiasi pascatranslasi;4; ampir semana #erpa glioprotein"
ecali al#min;
+; Bersifat polimorfisme ciri #a1aan pdpoplasi dgn sediitna 2 macam fenotipe3;
conto$> gol; @ara$ BO
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Plasma Proteins
• ore t$an 200
• ost a#ndant
– l#min - +-, g
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#lumin
- erpaan protein tama dalam plasma;
- emperta$anan ,-80% teanan osmoti;
- Berfngsi mengiat #er#agai macam ligand"
seperti> asam lema #e#as" Ca" C" Hn"
$ormon steroid" #ilir#in" met$eme
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#lumin
- l#min /ga dapat mengiat o#at-an" seperti> slfonamid" penisilin-5"
dimarol" aspirin
- Penait $epar aan memperli$atan rasio
al#min
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Trans"erin
• dala$ β?-glo#lin #er#ent glioproteinang disintesis di $epar;
• Berfngsi se#agai alat transpor #esi *e43 nt di#a1a e /aringan;
• Gia #esi tida diiat ole$ transferin" maaaan men/adi proosidan;
C l l i
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Cerulolasmin
• Protein ini adala$ α2-glo#lin angmengandng 90% C plasma;
7etapi ?0% C teriat longgar pd al#min"
se$ingga mda$ dilepas e /aringan;
• Cerloplasmin mengandng ferrosidase
ang mengatalisis ion *e2 --K *e4"
arena $ana ion *e4 ang mamp
#eriatan dgn apotransferin;
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Imuno&loulin Plasma
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Imuno&loulin Plasma
• @isintesis dalam sel plasma;
• el plasma adala$ trnan el-β angmensintesis dan menseresian imno-
glo#lin se#agai respon ter$adap pa/anan
#er#agai antigen;
• ema imnoglo#lin mengandng paling
rang 2 rantai ringan dan 2 rantai #erat;
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Classes o" Immuno&loulins
•Dg5 ! Ddentifies microorganisms forenglfment or lsis
• Dg& ! Dn$i#its parasite in.asion> in.ol.ed inallergic reactions
• Dg@ ! Inno1n• Dg ! Basis for passi.e immnit pro.ided #
#reast mil" aggltinates infectios agents insecretions otside t$e #od" present in tears"
mcos• Dg ! Ddentifies microorganisms for
englfment or lsis
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• Basic + c$ain strctral nit – W = 2),,000 2)2000 = ?60000
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!irino&en
• Coaglation• trctre:
–W 4+0 000
–eNence of amino acids is no1n40003
–+" 4 strctre
• 6 polpeptide c$ains" 2α 6"0003"2β ,6"0003" 2γ +"0003
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!unction>
–Blood coa&ulation ;clottin&=
Fibrinogen Fibrin
Thrombin
Fibrin Degradation (FD!lasmin
lasmin is end produ"t o# #ibrinol$ti" s$stem
%lot needs to be remo&ed'ot needed #ore&er%ould embolie to lungs) brain
lasmin is end produ"t o# #ibrinol$ti" s$stem%lot needs to be remo&ed
'ot needed #ore&er%ould embolie to lungs) brain
Hato&loin
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Hato&loin
• erpaan glioprotein plasma ang
mengiat $emoglo#in estraorpslar;
• em#ent omple #-p emoglo#in-
aptoglo#in3;
• # estraorpslar merpaan $asil
pengraian ?0% # ang dilepas e dlm
sirlasi;
Keusta*aan
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Keusta*aan
• ars" @B;" ars" @;" mit$ C; ?996; Basic medical
biochemistry: a clinical approach; @alam: B;I; Pendit"pener/ema$; Bioimia Eedoteran @asar: e#a$Pendeatan Elinis; &ds; G; ono;" ; adiin;" ;D;andera; Gaarta: &5C" 2000: 6?2 - +;
• rra" (E; 2004; Porfirin dan pigmen emped; @alam: ndr artono" pener/ema$; Harper’s Biochemistry. 2,t$ed; &ds; (;E; rra" @;E; 5ranner" P;; aes" ;W;(od1ell; c5ra1-ill Companies" 'e1 or: 4+2 - 9;
• c$mm" @&; ?992; Essentials of biochemistry ; @alam:oc$; adiin" pener/ema$; Dntisari Bioimia; Gaarta:Bina sara" ?994: ?+;
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K t *
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Keusta*aan
•
ar#t" C; ?,0 Blood; @o1nload ?9-?0-20?0;$ttp: