Ha hc Hemoglobin

M uChc nng chnh ca hng cuVn chuyn O2 t phi ti mVn chuyn CO2 t m ti phi

Hng cu m nhn c chc nng ny nh c hemoglobin (Hb) Hb chim 34% hng cu, #15g/ 100ml

M uHemoglobin (Hb) l chromoprotein, TLPT #68.000, hnh bu dc 5,5 X 6,5 X 5,5 nmCu trc gm 2 phn:Nhm ngoi: HemProtein: GlobinTng hp Hb bt u t cc tin nguyn hng cu65% giai on nguyn hng cu35% giai on hng cu li

Tng hp Hemoglobin

Cu trc HemProtoporphyrin IX kt hp vi Fe++Tt c cc vng pyrol u nm trn mt phng ca HemSt lin kt vi 4 vng pyrol qua 4 nguyn t N. Lin kt th 5 vi His on xon F8. Lin kt th 6 vi Oxy khi Hb mang oxy (Oxy nm gia St v His on xon E7)

Tng hp globin

Tng hp globinCc loi globin khc nhau kt hp vi hem to thnh cc hemoglobin khc nhau.Globin gm 4 chui/1 phn t HbGlobin gm 2 loi: Loi beta gm 146 aa: b, g, d v e. b- cluster (cc gen b, g, d v e globin ) trn nhnh ngn ca NST 11 Loi alpha gm 141 aa: a v z. a- cluster (cc gen a v z globin) trn nhnh ngn ca NST 16

Globin gene clusters

Tng hp globinTng hp Globin bt u t tun th 3 ca thai kPhi thai Hemoglobin Gower I ( z2e2) Hemoglobin Portland ( z2g2) Hemoglobin Gower II (a2e2)Bo thai : HbF (a2g2), HbA (a2b2)Ngi ln : HbA, HbA2 ( a2d2), HbF.

S tng hp cc chui globin qua cc giai on pht trin

Cc chui Alpha & beta

GlobinMi chui c 8 on xon, k hiu t A n H, xon alpha quay phi, chim 70%.Cc on gp khc gia cc on xon.Mi chui c cu trc bc 3 hon chnh. S t hp ca 4 chui to cu trc bc 4.Cc chui globin c ch lm gia, nhn hem c vi trong .Deoxyhemoglobin cn gn 2-3 DPG khong trng trung tm.

Hemoblobin ngi trng thnh

Hb AHb A2Hb FCu trca2b2a2d2a2g2T l %96-98 %1.5-3.2 %0.5-0.8 %

Hemoglobin A

Cc chc nng ca HemoglobinVn chuyn Oxygen ti mPhn ng ca Hb & oxygenOxygen ha ch khng phi l oxy haMt Hb c th gn vi 4 phn t O2 Cn di 0.01 giy cho oxygen haCc chui b xch gn nhau hn khi b oxygen haKhi gn oxy 2,3-DPG b y ra ngoiCc chui b cch xa nhau khi O2 gii phng, cho php 2,3-DPG i vo lm gim i lc ca O2 vi Hb

Oxy & deoxyhemoglobin

ng cong phn ly Oxygen-hemoglobinKh nng vn chuyn O2 ca Hb khc nhau cc Po2 khc nhauDng ch S (Sigmoid shape)Gn vi 1 phn t lm thun vic gn cc phn t tip theo

P 50 (p lc ring phn ca O2 ti Hb bo ha mt na O2) 26.6mmHg

ng cong phn ly Hb-oxygen

ng cong phn ly Hb-oxygenHnh dng bnh thng ca ng cong ph thuc vo:

Nng 2,3-DPGNng H+ (pH)CO2 trong hng cuCu trc ca Hb

ng cong phn ly Hb-oxygenDch sang phi (d dng phn ly oxy)2,3-DPG caoH+ caoCO2 caoHbSDch sang tri (kh gii phng oxy )2,3-DPG thpHbF

Chc nng m ca HbHb gp phn iu ha thng bng acid- baseT chc KHbO2 KHb + O2 KHb + H2CO3 KHCO3 + HHb+ Ti phi

HHb+ + O2 HHbO2 HHbO2 + KHCO3 H2 CO3 + KHbO2 H2O CO2