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8/18/2019 Kp 1 2 2 Komposisi Darah

1/57

BLOOD COMPOSITION

dr. Husnil Kadri, M.Kes

Biochemistry DeartementMedical !aculty O" #ndalas $ni%ersity

Padan&


2/57

BIOSINTESIS HEMOGLOBIN(PORFIRIN)


3/57

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


4/57

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


5/57

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.

5


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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.

7


8/57

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


9/57

,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

9


10/57

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


11/57

!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


12/57

!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


13/57

!un&si $tama Darah

6. Pen&an&*utan hormon 1en&aturan metaolisme

7. Pen&an&*utan metaolit

'8. Koa&ulasi


14/57

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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16/57

Ph i l Ch t i ti "


17/57

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


18/57

Components of Blood

–33< lasma

–

23< cells•77< (BCs

•? '< 9BCs and latelets


19/57


20/57

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


21/57

*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


22/57

&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


23/57

&rt$roctes (BCs3

Figure 17.3


24/57

&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


25/57

trctre of emoglo#in

Figure 17.4

H l i


26/57

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


27/57

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


28/57

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


29/57

@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


30/57

'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


31/57

&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


32/57

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


33/57

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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35/57

• 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


36/57

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


37/57

&migration F P$agoctosis in WBCs


38/57

$NS$( 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


39/57

•

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


40/57

Protein Plasma

- Bagian tama nsr padat dalam plasma;

- Eonsentrasi total protein plasma -",

g

?; alting-ot 'a2O+ 24%" dll3

2; &letroforesis


41/57

Hone &lectrop$oresis of Plasma

Proteins

- +

pI6.0 5.6 5.1 4.7

globulins albumin

γ β α

α


42/57

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


43/57

Plasma Proteins

• ore t$an 200

• ost a#ndant

– l#min - +-, g


44/57

#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


45/57

#lumin

- l#min /ga dapat mengiat o#at-an" seperti> slfonamid" penisilin-5"

dimarol" aspirin

- Penait $epar aan memperli$atan rasio

al#min


46/57

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


47/57

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;


48/57

Imuno&loulin Plasma


49/57

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;


50/57

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


51/57

• Basic + c$ain strctral nit – W = 2),,000 2)2000 = ?60000


52/57


53/57

!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


54/57

!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


55/57

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


56/57

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: ?+;

56

K t *


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Keusta*aan

•

ar#t" C; ?,0 Blood; @o1nload ?9-?0-20?0;$ttp:

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