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00HßR0CK At0 SClAttA

ßl000 6R0uPlt6 SYSTFH
Croup # 3
Croup Members
W Marlna 8rldgemohan
W nemlsscla !ordan
W uevanand 8amnarlne
W 1lffany klng
uLllne
W uombrock blood grouplng sysLem
PlsLory
ÞroperLles
ÞopulaLlon ulsLrlbuLlon
ÞhenoLype ulsLrlbuLlon
Cllnlcal Slgnlflcance
8lochemlsLry
uLllne
W Sclanna blood grouplng sysLem
ClasslflcaLlon
PlsLory
ÞroperLles
Cllnlcal Slgnlflcance
ÞopulaLlon ulsLrlbuLlon
ÞhenoLype ulsLrlbuLlon
8lochemlsLry
uombrock 8lood Crouplng SysLem
W 1he uombrock blood group sysLem ls Lhe classlflcaLlon
of human blood based on Lhe presence of cerLaln
glycoproLelns called glycosylphosphaLldyllnoslLol (CÞl)Ŵ
anchored proLelnŦ
W 8epresenLed by Do
W (uo) anLlgen was flrsL dlscovered on Lhe surface of 88C
uombrock AnLlgens
1he assoclaLed anLlgens of Lhls blood group
sysLem areť
W uo
a
W uo
b
W Cy
a
(Cregory anLlgen)
W Py (Polley anLlgen)
W !o
a
(!oseph anLlgen)
PlsLory
W AnLlbodles Lo Lhe uombrock anLlgen uo
a
were
dlscovered ln 1963 ln a paLlenL who had
recelved a blood LransfuslonŦ
W ln 1973 a second uombrock anLlgen (uo
b
) was
ldenLlfledŦ
PlsLory ConL'd
1he uombrock blood group was expanded ln Lhe
1990s
lncluded Lhree oLher anLlgens namelyť
W Cy
a
(Cregory anLlgen)
W Py (Polley anLlgen)
W !o
a
(!oseph anLlgen)
nŦ8 All occurred on Lhe same proLeln as Lhe uo
anLlgensŦ
ÞroperLles of Lhe uombrock AnLlgen
W lL ls encoded by Lhe gene @(AuÞ
rlbosylLransferase 4)
W ocaLed on Lhe shorL arm of chromosome
12p13Ŧ2Ŵp12Ŧ1
ÞroperLles ConL'd
W 1he CÞl ls embedded ln Lhe membrane of
uombrockŴexpresslng cells hereby enabllng Lhe
proLeln and lLs anLlgenŴconLalnlng resldue Lo
remaln exposed on Lhe ouLslde surface of Lhe
cells
W number of amlno aclds ls 314
W CeneLlc varlaLlons ln @LhaL resulL ln an
alLered amlno acld sequence of Lhe encoded
proLeln glve rlse Lo Lhe dlfferenL uombrock
Carrler ÞroLeln
ÞopulaLlon ulsLrlbuLlon
W SLudles lndlcaLed LhaLť
W 63 Ʒ of norLhern Luropeans carry Lhe uo
a
anLlgen
W uo

anLlgen has an lncreased lncldence ln
Afrlcans and Aslans
W Cy
a
ţ Pyţ and !o
a
anLlgens have been esLlmaLed
Lo occur ln more Lhan 99 Ʒ of lndlvlduals
ÞhenoLype ulsLrlbuLlon
9°otyp Do
a
Do
b
Gy
a
ny Io
a
Caucas|a°s 8|acks
Do (a+bŴ) + 0 + + + 18 11
Do (a+b+) + + + + + 49 44
Do (aŴb+) 0 + + + + 33 43
Gy (aŴ) 0 0 0 0 0 8are 8are
nyŴ 0 wk wk 0 0/wk 0 8are
Io (aŴ) wk 0/wk + wk 0 0 8are
uombrock AC ulsLrlbuLlon ln 8ody
AparL from Lhe surface of Lhe 88C uo
a
ls found
onť
W ymphocyLes and lymph nodes
W 8one marrow
W 1lssues of Lhe spleenţ ovarlesţ LesLesţ
lnLesLlnesţ and feLal hearLŦ
nŦ8 1he expresslon of uombrock anLlgens ls
null ÞhenoLype
W 1he absence of uombrock anLlgens occur
because of loss of CÞlŴanchored proLelns from
Lhe surface of 88C resulLlng ln uombrockŴnull
phenoLype
W oss of Lhe proLeln resulLs ln Þaroxysmal
nocLurnal Pemogloblnurla (ÞnP) Ŷ (88C
undergo premaLure desLrucLlon by lmmune
cells )
AnLlbodles and Cllnlcal Slgnlflcance
W uombrock anLlbodles ls predomlnanLly lgC
W uo noL acLlvaLe complemenL sysLem
W 1hey have noL been assoclaLed wlLh cllnlcal
PemolyLlc ulsease of newborn (Pun)
W Powever anLlbodles have been assoclaLed
wlLh severe PemolyLlc 1ransfuslon 8eacLlon
(P18)
8lochemlsLry
W 8eslsLanL Lo papaln or flcln and chloroqulne
W SenslLlve Lo Lrypslnţ ĐŴchymoLrypslnţ pronaseţ
or u111200
Summary
81 Nam
(81
Numb)
Comosom
Locat|o°
G° Nam
GN (81)
9|ct
1opo|ogy
(Numb of
am|°o ac|s)
Compo°°t
Nam (CD
°umb)
Assoc|at
8|oo Goup
A°t|g°s
Dombock
(014)
12p13Ŧ2Ŵp12Ŧ1 u (u)
CÞlŴllnked
(314)
uo
glycoproLelnŤ
A81 4
uo
a
ţ uo
b
ţ Cy
a
ţ
Pyţ !o
a
SClAttA SClAttA ßl000 6R0uPlt6 ßl000 6R0uPlt6
SYSTFH SYSTFH
Sclanna 8lood Croup SysLem
A polymorphlc blood group anLlgen LhaL are
deLermlned by eplLopes on Lhe surface of red blood
cellsŦ
Sclanna 8lood Croup conslsLs of anLlgensť
W Sc1
W Sc2
W Sc3
W 8d ( 8adln)
Sclanna 8lood Croup SysLem
LsLabllshed 1974
W lS81Ŵ SC
W lS81 #Ŵ 013
W Lher nameŴ Sc
Lxpresslon
W Lher blood cellsť Weakly expressed ln
leukocyLesţ Lhymusţ lymph nodesţ spleen
W 1lssueť leLal llverţ bone marrow ln adulLs
ClasslflcaLlon
1hese anLlgens are furLher classlfled as by Lhelr
frequenclesŦ
W Sc2 and 8d are consldered as low frequency
W Sc1 and Sc3 are boLh hlgh frequency
PlsLory
W ln 1962ţ an anLlbody Lo a hlghŴprevalence
anLlgenţ anLlŴSmţ was descrlbed by SchmldLŦ
W 1963 low lncldence anLlbody (anLlŴSc2/8u
a
)
was dlscovered by Anderson
PlsLory
W 8d (8adln) was recenLly added Lo Lhe Sclanna
sysLemŦ Powever lL was descrlbed by Lhe
expresslon of Sclanna sysLem anLlgens on
L8MAÞŦ
W 8d ls Lhe resulL of a muLaLlon ln íkMAl LhaL
produces a change ln Lhe expressed proLeln
(Þro60Ala)Ŧ
W lmmunobloLLlng wlLh anLlŴ8d had shown LhaL
ÞroperLles of Lhe anLlgen
W 1he anLlgens are carrled on human eryLhrold
membraneŴassoclaLed proLeln (L8MAÞ)Ŧ
W ConslsL of 473 amlno aclds
Carrler ÞroLeln
AssoclaLed gene
W Sclanna glycoproLeln ls Lhe anLlgen sLrucLure
LhaL encodes Lhe SC geneŦ
W ocaLed on chromosome 1 p34
AnLlbody and Cllnlcal Slgnlflcance
W lgC ls Lhe respecLed anLlbody for Sclanna 8lood group
W AnLlbodles are produced because of 88C sLlmulaLlon
Lhrough Lransfuslon or pregnancy
W Can acLlvaLe complemenL sysLemŦ
W Pave been reporLed Lo cause mlld (Pun)Ŧ
Cllnlcal Slgnlflcance
W lL ls noL known Lo cause cllnlcal Lransfuslon problemsŦ
W AnLlŴSc1 ls a rare lgC anLlbody reacLlve by Lhe anLl
globulln LesLŦ
Slbllngs of paLlenLs maklng Lhe anLlbody should be LesLed
for compaLlblllLyţ and paLlenLs encouraged Lo donaLe blood
for long Lerm sLorage when Lhelr cllnlcal condlLlon permlLsŦ
Cllnlcal Slgnlflcance
W Slnce Lhe Sc2 anLlgen has a lowŴlncldenceţ Lhe greaL
ma[orlLy of donor blood samples wlll be compaLlbleŦ
W AnLlŴSc2 ls usually lgC and reacLlve by Lhe anLl globulln
LesLŦ AbouL 1Ʒ of random donor red cell samples are
Sc2+Ŧ
ÞopulaLlon ulsLrlbuLlon
W nţ a hlgh frequency anLlgen found ln greaLer
Lhan 99 Ʒ of mosL populaLlonsŦ
W 1he frequency of Sc2 ls abouL 1Ʒ of norLhern
Luropeans buL Lhe frequency ls much lower ln
oLher populaLlons
W Scť1ţ2 ls more common ln MennonlLe
W nţ a hlgh frequency anLlgen found on all
cells excepL ln persons who Lype ScťŴ1ţŴ2
ÞhenoLype ulsLrlbuLlon
ÞhenoLypes Caucaslans 8lacks
Scť lţŴ2 99 100
Scť lţ 2 1 0
Scť Ŵlţ 2 very rare 0
Scť lţ Ŵ2ţ 8d+ very rare very rare
Scť lţ 2ţ 8d+ very rare 0
8lochemlsLry
W 1he Sc1 and Sc2 anLlgens can be LreaLed wlLh
u11
W 8eslsLanL Lo flcln/papalnţ LrypslnţrŴ
chymoLrypsln and ChloroqulneŦ
W 1he 8d anLlgen dlffers ln LhaL Lhe effecL of
Lrypsln and áŴ chymoLrypsln LreaLmenL ls
varlableŦ
Summary
81 Nam
(81
Numb)
Comosom
Locat|o°
G° Nam
GN (81)
9|ct
1opo|ogy
(Numb of
am|°o ac|s)
Compo°°t
Nam (CD
°umb)
Assoc|at
8|oo Goup
A°t|g°s
c|a°°a
(013)
1p34 L8MAÞ (SC) 1ype l (473) L8MAÞ
Sc1ţ Sc2ţ Sc3ţ
8d
8eferences
W karaţ kŦ8Ŧţ 2010Ŧ 1he Puman 8odyţ lbysl–y oo cltnotloţ 1
sL
edŦţ
8rlLannlca LducaLlonal Þubllsherţpp110Ŵ111Ŧ
8eLrleved hereť
hLLpť//booksŦgoogleŦcom/books?ldƹlbZwlu1mMCƎpgƹÞA110Ǝdqƹdombrock+
blood+grouplng+sysLemƎhlƹenƎelƹ3_qv1u2oPZPqgCe![dPCACƎsaƹxƎolƹbook_
resulLƎcLƹresulLƎresnumƹ2Ǝvedƹ0CukC6ALwAC#vƹonepageƎqƎfƹfalse
W !ohn Þţ Ceerţ eL alŦţ 2009Ŧ WlnLrobe's Cllnlcal PaemaLologyţ 12
Lh
edŦţ
lpplncoLLţpp 672Ŧ
8eLrleved hereť
hLLpť//booksŦgoogleŦcom/books?ldƹ68enzuu78vgCƎpgƹÞA644Ǝdqƹdombrock+bl
ood+grouplng+sysLemƎhlƹenƎelƹ3_qv1u2oPZPqgCe![dPCACƎsaƹxƎolƹbook_re
sulLƎcLƹresulLƎresnumƹ3Ǝvedƹ0Cu0C6ALwAg#vƹonepageƎqƹdombrockƷ20blo
odƷ20grouplngƷ20sysLemƎfƹfalse
8eferences
W uelores Malloryţ uŦMţ eL alŦţ 2003Ŧ lmmunohemaLologyţ too €
b –t½s set–y oo enotlo ţ vol 21ţ Lhe Amerlcan 8ed
Crossţ pp 70Ŧ
8eLrleved hereť
hLLpť//a1881ŦgŦakamalŦneL/7/1881/26640/v0001/redcrossŦdownloadŦa
kamalŦcom/26640/pubs/lmmuno/21_2_03Ŧpdf
W hLLpť//scarfexŦ[oveŦprohosLlngŦcom/blood/13ŦhLml
W
hLLpť//blood[ournalŦhemaLologyllbraryŦorg/conLenL/96/7/262
1Ŧfull

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