红细胞与贫血-ppt课件

1、根底血根底血 液液 学学红细胞与贫血红细胞与贫血Erythrocyte & Anemia夏 立病理生理学教研室BLOODPlasma-55%Formed elements-45%Buffy coat-3%) are released into the blood prematurely.1. Erythropoiesis骨髓红系定向细胞增殖及其最重要受体的实际模型EPO：促红细胞生成素；GM-CSF：粒-巨核细胞集落刺激因子；IL3：白介素3； Transferrin: 转铁蛋白；Fibronectin:纤连蛋白1. ErythropoiesisToo fe

2、w RBCs leads to tissue hypoxiaRegulation of Erythropoiesis1. ErythropoiesisKidney (and liver toa smaller extent)releases ERYTHROPOIETIN. Erythropoietinstimulates redbone marrow. Enhancederythropoiesisincreases RBCcount. O2- carryingability of bloodincreases.Homeostasis: Normal blood oxygen levels St

3、imulus:Hypoxia (low bloodO2- carrying ability)due to Decreased RBC count Decreased amountof hemoglobin Decreased availability of O212345IMBALANCEIMBALANCEHypoxia Inducible Factor (HIF)Regulation of Erythropoiesis1. ErythropoiesisErythropoietin (EPO促红细胞生成素)34,000 Da, GlycoproteinReleased by the kidne

4、y in response to hypoxia (hemorrhage出血, hemolytic溶血, iron deficiency or high altitudes)Regulation of Erythropoiesis1. ErythropoiesisRegulation of Erythropoiesis1. ErythropoiesisErythropoietin (EPO)Direct stimulus for erythropoiesisMore rapid maturation of committed bone marrow cellsIncreased circula

5、ting reticulocyte count in 12 days Regulation of Erythropoiesis1. ErythropoiesisBalance between RBC production and destruction depends onHormonal controls (Erythropoietin, EPO) Adequate supplies of Folic Acid (folate叶酸)Vitamin B12, cyanocobalamin氰钴胺Amino AcidsIronVitamin B6, pyridoxine吡哆醇Ascorbic Ac

6、id (Vitamin C)Regulation of Erythropoiesis1. Erythropoiesis1. Erythropoiesis (Disease) Aplastic Anemia ( AA, 再生妨碍贫血 )1. Erythropoiesis (Disease)The marrow biopsy section of a young adult with very severe aplastic anemiaA normal marrow biopsy section of a young adult.获得性再生妨碍性贫血是一种血液中红细胞、中性粒细胞、单核细胞和血小

7、板减少，而骨髓组织被脂肪组织替代、造血祖细胞几乎缺如的临床综合征。病因：1、毒性物质：苯、有机磷2、病毒感染：EB、肝炎病毒、HIV3、药物：氯霉素、噻氯匹定4、本身免疫病：红斑狼疮5、妊娠共同发病途径：细胞毒T细胞本身反响An immune attack of Tc cells targets hematopoietic stem and progenitor cells (CD34+ cells) apoptosis hematopoietic failureAplastic Anemia ( AA, 再生妨碍贫血 )1. Erythropoiesis (Disease)Anemia of

8、 Chronic Renal Disease (慢性肾脏疾病的贫血) 病变的肾脏产生EPO减少是贫血的主要缘由 毒性代谢产物的累积导致红细胞寿命缩短、骨髓抑制 并发炎症或营养不良可加重贫血慢性肾脏疾病患者血细胞比容与内生肌酐去除率的关系。尿毒症患者的循环血EPO程度下降 正常个体和单纯贫血患者有肾的尿毒症患者无肾的尿毒症患者1. Erythropoiesis (Disease)Anemia of Chronic Renal Disease (慢性肾脏疾病的贫血)A.外周血涂片慢性肾脏疾病没有特征性的红细胞改动。偶见红细胞碎片，靶形红细胞，棘形红细胞等异常红细胞形状。B.外周血涂片溶血尿毒症综合

9、征。大量的破碎红细胞是溶血尿毒症综合征的典型表现。红细胞大小不一和少量球形红细胞。1. Erythropoiesis (Disease)2. Cell MembraneFlexibility to change shapeThe red cell must be able to change shape and squeeze through small capillaries. Sphingomyelin (SM 鞘磷脂)Phosphatidylcholine (PC磷脂酰胆碱)Phosphatidylethanolamine (PE 磷脂酰乙醇胺)Phosphatidylserine (P

10、S 磷脂酰丝氨酸)2. Cell MembraneThe red cell membrane consists of a bipolar lipid layer supported by structural proteins. Protein, 49%Lipid, 42%Carbohydrate, 8%Inorganic ion, 1% Outermost layer: glycolipids, glycoproteins Central layer: cholesterol, phospholipids Inner layer: cytoskeleton (骨架蛋白) Spectrin 血

11、影蛋白 Ankyrin锚蛋白ProteinsIntegral proteins (I， 内在蛋白) Band 3区带3蛋白，阴离子交换蛋白 Glycophorin 血型糖蛋白are important for the active transport of solutes across the membranePeripheral Proteins (P， 周围蛋白) Spectrin 血影蛋白 Actin肌动蛋白 Ankyrin锚蛋白 Band 4 区带4蛋白membrane skeleton on the inner surfaceto maintain the biconcave sha

12、pe of the red cellto strengthens the membrane against sheer force2. Cell MembraneProteinsIntegral proteins (I， 内在蛋白) Band 3区带3蛋白，阴离子交换蛋白 Glycophorin 血型糖蛋白are important for the active transport of solutes across the membranePeripheral Proteins (P， 周围蛋白) Spectrin 血影蛋白 Actin肌动蛋白 Ankyrin锚蛋白 Band 4 区带4蛋白

13、membrane skeleton on the inner surfaceto maintain the biconcave shape of the red cellto strengthens the membrane against sheer forceProteins in the red cell membrane can be solubilised by a detergent called sodium dodecyl sulphate (SDS) and then be separated according to their size using polyacrylam

14、ide gel-electrophoresis (SDS-PAGE).2. Cell MembraneLipids60% phospholipid 磷脂30% neutral lipids (mainly cholesterol胆固醇)10% glycolipids糖脂The phospho- and glyco-lipids are structural with polar groups (hydrophilic) on the external and internal surfaces of the cell.Non-polar groups (hydrophobic) form a

15、barrier at the centre of the membrane.Asymmetry of membrane phospholipids膜磷脂的非对称分布2. Cell MembraneSphingomyelin (SM 鞘磷脂)Phosphatidylcholine (PC磷脂酰胆碱)Phosphatidylethanolamine (PE 磷脂酰乙醇胺)Phosphatidylserine (PS 磷脂酰丝氨酸)Carbohydratesare mostly found on the external surface of the red cell membrane. Monos

16、accharides 寡糖are associated with specific blood group antigens, e.g. ABH and Lewis.A gene product(A transferase)GalGlcGalGlcNAcRed cell membraneType 2 precursorNote: 14 linkageband 31234Fuc1234GalNAcDonor nucleotide(UDP-GalNAc)Acceptor sugar(Galactose)Specific 13linkageProducing blood group A antige

17、n on the red cell of a group A individual. 2. Cell MembraneMembrane Functioncellular deformability and membrane stabilitymembrane permeabilitymembrane antigenicity and immunologic functionreceptors2. Cell MembraneMembrane Functioncellular deformability and membrane stability structural characteristi

18、cs - Biconcave shape is the contribute to gas transport huge surface area relative to volumeshorten the distance of gas exchange2. Cell MembraneMembrane Functionmembrane permeability2. Cell Membrane红细胞膜的通透性，人红细胞主要的离子转运途径AE1, band 3 anion exchanger; 带3阴离子运转蛋白 高丰度跨膜糖蛋白，转运Cl- & HCO3-AQP1, water cha

19、nnel aquaporin 1;水通道蛋白KCC1, 氯化物-阳离子协同转运蛋白家族KCl协同转运系统 NKCC2, Na-K-Cl协同转运蛋白基底侧分子方式SK, small conductance potassium channel.小电导钾通道2. Cell MembraneMembrane Functionmembrane antigenicity and immunologic function Blood group antigens are associated with the red cell membrane and are either integral to its

20、structure or are adsorbed onto it from the plasma.They are made of proteins or carbohydrates. senescent cell antigen, SCA老化抗原2. Cell MembraneANTIGENS ON RBC MEMBRANEMembrane Functionmembrane antigenicity and immunologic function2. Cell MembraneMembrane Functionreceptorshormone receptors：insulin，gluc

21、agonNeurotransmitter receptors：noradrenalineVirus receptors：flu. receptorothers：TfR，EPO receptor2. Cell Membrane2. Cell Membrane (disease)Hereditary Spherocytosis, Elliptocytosis遗传性球形红细胞增多症，椭圆形红细胞增多症abnormal spectrin gene reduced spectrin synthesis or dysfunctional spectrin spherocytes or elliptocyt

22、es遗传性球形红细胞增多症是一种以外周血涂片成熟红细胞呈球形、网织红细胞增多和脾脏肿大为特征的先天性溶血性贫血。红细胞在经过脾脏微循环时，由于细胞膜丧失和膜面积减少而呈球形样改动并伴有变形可塑性降低。失去变形才干的红细胞在脾脏被破坏最终导致贫血。Hereditary Spherocytosis, Elliptocytosis遗传性球形红细胞增多症，椭圆形红细胞增多症遗传性球形红细胞增多症病理生理学。HS主要缺陷为细胞膜丧失，膜面积减少，外表积减少，导致细胞球形变，变形才干减弱。2. Cell Membrane (disease)the process of spherocytosis in h

23、ereditary spherocytosisHereditary Spherocytosis, Elliptocytosisreduced spectrin synthesisdysfunctional spectrinabnormal spectrin geneSpectrin malfunction within erythrocyte membraneErythrocytes are exposed to high sheer forces within the microcirculationCytoskeleton function impaired; cell loses abi

24、lity to deformSpherocyte: a small, more rigid, spherical erythrocyte resultsCells are either destroyed within the microcirculation or detected and removed by the reticuloendothelial system of the spleenHaemolysis; premature red cell death occurs causing anaemia2. Cell Membrane (disease)The Congenita

25、l Dyserythropoietic AnemiasCDA先天性红细胞生成异常性贫血先天性红细胞生成异常性贫血CDA是指一类稀有的，以红系无效造血，多核红细胞和组织内铁蓄积为特征的遗传性难治性贫血。可分为、型。先天性红细胞生成异常性贫血CDA型中带3蛋白-N-乙酰乳糖聚糖链合成缺乏，而鞘磷脂表现为过度糖基化。2. Cell Membrane (disease)LipidsPS exposure磷脂酰丝氨酸外翻磷脂酰丝氨酸外翻可启动凝血活化途径PS exposure to trigger thrombosis磷脂酰丝氨酸外翻可被巨噬细胞识别并吞噬PS exposure to be recogn

26、ized and phagocytized by macrophage2. Cell Membrane (disease)Alteration in lipid composition can produce target cells or acanthocytes.靶形红细胞棘形红细胞Lipids2. Cell Membrane (disease)3. Cell Metabolismmetabolic changes in erythropoiesis metabolic capabilitynormoblast reticulocyte mature erythrocyteMultipli

27、cation capacityDNA synthesizeRNA synthesizeRNA existenceProtein synthesizeHeme synthesizeLipoid synthesizeTricarboxylic acid cycleOxidative phosphorylationTHE EMBDEN-MEYERHOF PATHWAY (EMP)Pentose-phosphate shunt pathway 3. Cell Metabolism;3. Cell MetabolismHAEMOGLOBIN 血红蛋白 Haemoglobin, MW 68,000Da 9

28、6% of the red blood cells dry weight 65% of haemoglobin synthesis occurs during the nucleated stages of RBC maturation and 35% occurs during the reticulocyte stageHemegroup(a) Hemoglobin consists of globin珠蛋白 (two alpha and two beta polypeptide chains) and four heme血红素 groups.(b) Iron-containing hem

29、e pigment (a protoporphyrin ring 原卟啉环plus ferrous iron (Fe2+)a Globin chainsa Globin chainsb Globin chainsb Globin chainsHAEMOGLOBIN Structure3. Cell MetabolismHAEMOGLOBIN Function3. Cell MetabolismIron atom in each heme can bind to one O2 moleculeEach Hb molecule can transport four O2Hemoglobin bin

30、ds reversibly with oxygenO2 loading in the lungs Produces oxyhemoglobin氧合血红蛋白 (ruby red)O2 unloading in the tissues Produces deoxyhemoglobin脱氧血红蛋白 or reduced hemoglobin (dark red) CO2 loading in the tissues Produces carbaminohemoglobin 氨基甲酰血红蛋白(carries 20% of CO2 in the blood)HAEMOGLOBIN Globin珠蛋白3.

31、 Cell MetabolismHemoglobin Genes and Gene Products、Mehta, A. B., and A. V. Hoffbrand. 2000. Haematology at a glance, Blackwell Science, Malden, Mass.HbF: 2 and 2HbA1: 2 and 2HbA2: 2 and 2HbE: 2 and 2Yolk sacLiverSpleenBone marrow3. Cell MetabolismHemoglobin Genes and Gene ProductsHAEMOGLOBIN Globin珠

32、蛋白Embryonic haemoglobins include Gower 1, Gower 2 and Hb Portland.HbF is the predominant haemoglobin of fetal life (65-95%). Adults have only trace amounts of HbF (95%) and HbA2 (2.5-3.5%) are the main adult haemoglobins.3. Cell MetabolismSix Hb variants are normally formed. HAEMOGLOBIN Globin珠蛋白3.

33、Cell MetabolismHemin can accelerate the synthesis珠蛋白的合成受血红素调理HRI，Heme-regulated eIF2 kinase；eIF，eukaryote Initiation factor；eIF-2B ，eIF-2B GDP-GTP exchange factorHAEMOGLOBIN Globin珠蛋白(卟啉)吡咯环次甲基HAEMOGLOBIN HEME血红素3. Cell MetabolismHAEMOGLOBIN HEME血红素3. Cell MetabolismHEME consists of four pyrrole rin

34、gs 吡咯环with a central iron atom linked to the four nitrogen atoms.The iron atom has two further binding sites, one of which is bound to a globin histidine residue and the other binds reversibly to oxygen. 3. Cell MetabolismHAEMOGLOBIN HEME血红素血红素合成的限速酶：ALA synthase， aminolevulinic acid合酶 (-氨基-酮基戊酸合酶)辅

35、酶：磷酸吡哆醛Vit B63. Cell MetabolismRegulation of ALA Synthase ALA合酶的调理 Level of enzyme synthesis ALA合酶的表达程度Enzyme synthesis, as well as its transport to the mitochondria, is inhibited by elevated levels of heme and hemin (the Fe3+ oxidation product of heme) 过量的血红素高铁血红素抑制酶合成Enzyme synthesis is upregulate

36、d by a large number of drugs including barbiturates, steroids (e.g. testosterone) and some oral contraceptives. 巴比妥类药物，类固醇，睾丸素，口服避孕药Level of enzyme activity ALA合酶的酶活性Heme 血红素and hemin高铁血红素 inhibit ALA synthase activityRequires pyridoxal phosphate (Vitamin B6，磷酸吡哆醛) as a coenzymeHAEMOGLOBIN HEME血红素3.

37、 Cell Metabolism (disease)PORPHYRIAS卟啉症卟啉病是由于血红素生物合成途径中酶缺陷导致反响过程中间代谢产物过量生成与蓄积，并引发神经系统病症和或照顾性皮肤病症等疾患血红素生物合成途径中各种酶与中间代谢产物，以及与酶缺乏相对应的卟啉病类型。IronProtoporphyrinGlobinHaemHaemoglobinThalassaemiaIron deficiencyChronic inflammationMalignancyChronic infections and inflammatory disorders cause chronic anaemia

38、as a result of;1. slightly shortened red blood cell life span 2. sequestration of iron in inflammatory cells called macrophagesBoth procedures result in a decrease in the amount of iron available to make red blood cells. 3. Cell Metabolism (disease)HEMOGLOBINOPATHY 血红蛋白病血红蛋白病是全球最常见的遗传性红细胞疾病。是指珠蛋白生成妨

39、碍贫血珠蛋白合成缺乏和异常血红蛋白病珠蛋白一级构造中氨基酸突变，以地中海贫血和镰状细胞综合征为主。可导致中度到重度贫血。3. Cell Metabolism (disease)地中海贫血以一条或多条珠蛋白链合成缺陷的遗传性疾病。包括和-地中海贫血。由于珠蛋白链合成的不平衡，导致无效红细胞生成，溶血和不同程度的贫血。HEMOGLOBINOPATHY-Thalassemias 地中海贫血 3. Cell Metabolism (disease)HEMOGLOBINOPATHY-Thalassemias 地中海贫血 地中海贫血，过多的 链构成 4分子HBH可溶-地中海贫血，过多的链不可溶致红细胞损伤

40、，呵斥重度贫血。3. Cell Metabolism (disease)HEMOGLOBINOPATHY-Thalassemias 地中海贫血 3. Cell Metabolism (disease)HEMOGLOBINOPATHY-Sickle cell anemia (镰状细胞贫血)3. Cell Metabolism (disease)镰状细胞综合征是-珠蛋白链第6位氨基酸谷氨酸被缬氨酸所替代，导致血红蛋白分子脱氧时变得不可溶，构成HBS多聚体，改动了红细胞的正常构造，使红细胞呈生硬，并由双凹圆盘状变为镰刀形。这类红细胞变形才干差，易破损溶血，导致血管阻塞、组织缺氧、损伤、坏死。Gene

41、tics of Sickle Cell AnemiaHEMOGLOBINOPATHY-Sickle cell anemia (镰状细胞贫血)3. Cell Metabolism (disease)Hemoglobin E/hemoglobin-myoglobin.htmlHomozygous HbSNormal neonateNormal adultHbSCHeterozygous HbSRelative protein chargeStart (samples applied here)Anode (+)

42、Cathode (-)HEMOGLOBINOPATHY-Sickle cell anemia3. Cell Metabolism (disease)HEMOGLOBINOPATHY-Sickle cell anemia3. Cell Metabolism (disease)Schema summarizing the pathophysiology of sickle cell anemia. K+, potassium; NO, nitric oxide; PS, phosphatidylserine; RBC, red blood cell; ROS, reactive oxygen sp

43、ecies; TF, tissue factor; WBC, white blood cell; XO, xanthine oxidase.HEMOGLOBINOPATHY-Sickle cell anemia3. Cell Metabolism (disease)metabolic changes in erythropoiesis metabolic capabilitynormoblast reticulocyte mature erythrocyteMultiplication capacityDNA synthesizeRNA synthesizeRNA existenceProte

44、in synthesizeHeme synthesizeLipoid synthesizeTricarboxylic acid cycleOxidative phosphorylationTHE EMBDEN-MEYERHOF PATHWAY (EMP)Pentose-phosphate shunt pathway 3. Cell Metabolism;3. Cell MetabolismGlycometabolismMature red cells no mitochondrial apparatus for oxidative metabolism-Red cells deliver ox

45、ygen, not consume itLimited Glucose as a source of energyGlycolysis generates ATP and 2,3-bisphosphoglycerate 2,3-二膦酸甘油酸，2,3-DPGThe pentose phosphate pathway produces NADPH Glutathione synthesis (谷胱甘肽合成) the antioxidant defense system Energy required for: Maintenance of cation pumps Maintenance of R

46、BC integrity and deformability Reduce oxidants in the system Maintenance of HB in reduced state Maintenance of reduced sulfhydryl groups in HB and other proteins3. Cell MetabolismGlycometabolismKey Metabolic Pathways for the ErythrocyteGlycolysis or Embden-Meyerhof pathway 糖酵解，恩-迈氏途径Rapaport-Lueberi

47、ng Shunt2,3-DPG支路，2，3二膦酸甘油酸支路Hexose Monophosphate Shunt磷酸戊糖途径，磷酸己糖支路Methemoglobin reductase pathway 高铁血红蛋白复原酶途径3. Cell MetabolismKey Metabolic Pathways for the ErythrocyteGlycolysis or Embden-Meyerhof pathway糖酵解恩-迈氏途径3. Cell MetabolismKey Metabolic Pathways for the ErythrocyteGlycolysis or Embden-Me

48、yerhof pathway 糖酵解，恩-迈氏途径Generates 90- 95% of energy needed by RBCsGlucose is metabolized and generates two molecules of ATP (energy).Functions in the maintenance of RBC shape, flexibility and the cation pumps3. Cell MetabolismKey Metabolic Pathways for the ErythrocyteRapaport-Luebering Shunt 2,3-DP

49、G支路，2，3二膦酸甘油酸支路3. Cell Metabolism1,3-二膦酸甘油酸二膦酸甘油酸变位酶3膦酸甘油酸2，3-二膦酸甘油酸PO2 (mm Hg)Hb saturation (100%)5050CO2pH2,3-DPG CO2pH2,3-DPG Key Metabolic Pathways for the Erythrocyte3. Cell MetabolismRapaport-Luebering Shunt 2,3-DPG支路，2，3二膦酸甘油酸支路Key Metabolic Pathways for the Erythrocyte3. Cell Metabolismmfi.k

50、u.dk/PPaulev/chapter8/images/8-3.jpgRapaport-Luebering Shunt 2,3-DPG支路，2，3二膦酸甘油酸支路Key Metabolic Pathways for the ErythrocyteRapoport- Luebering Shunt 2,3-DPG支路，2，3二膦酸甘油酸支路Allows the RBC to regulate oxygen transport during conditions of hypoxia or acid-base imbalance.Permits the accumulation of 2,3-D

51、PG which is essential for maintaining normal oxygen tension, regulating hemoglobin affinity3. Cell MetabolismKey Metabolic Pathways for the Erythrocyte3. Cell MetabolismHexose Monophosphate Shunt 磷酸戊糖途径，磷酸己糖支路Key Metabolic Pathways for the Erythrocyte3. Cell MetabolismHexose Monophosphate Shunt 磷酸戊糖

52、途径，磷酸己糖支路Key Metabolic Pathways for the Erythrocyte3. Cell MetabolismHexose Monophosphate Shunt 磷酸戊糖途径，磷酸己糖支路Key Metabolic Pathways for the ErythrocyteMetabolizes 5-10% of glucose.NADPH is end productProtects the RBC from oxidative injury.If the pathway is deficient, intracellular oxidants cant be n

53、eutralized , and then globin denatures and precipitates. The precipitates are referred to as Heinz bodies.Most common defect is deficiency of the enzyme glucose-6-phosphate dehydrogenase (G-6PD，葡萄糖-6-磷酸脱氢酶).3. Cell MetabolismHexose Monophosphate Shunt 磷酸戊糖途径，磷酸己糖支路In red cells, there is a Met Hb red

54、uctase (高铁血红蛋白复原酶) which can use NADH to regenerate HbKey Metabolic Pathways for the ErythrocyteMethemoglobin reductase pathway 高铁血红蛋白复原酶途径3. Cell MetabolismOxy HbMet HbKey Metabolic Pathways for the Erythrocyte Maintains iron in the ferrous (Fe+) state. In the absence of the enzyme (methemoglobin r

55、eductase), methemoglobin accumulates and it cannot carry oxygen.3. Cell MetabolismMethemoglobin reductase pathway 高铁血红蛋白复原酶途径Key Metabolic Pathways for the Erythrocyte3. Cell Metabolism3. Cell Metabolism (Disease)Disorders of Red Cells Resulting from Enzyme Abnormalities酶异常导致的红细胞疾病Glucose-6-phosphat

56、e dehydrogenase (G6PD) deficiency 6-磷酸葡萄糖脱氢酶缺乏是最常见的红细胞酶缺陷，溶血发作只磷酸葡萄糖脱氢酶缺乏是最常见的红细胞酶缺陷，溶血发作只需在感染或运用需在感染或运用“氧化性药物，以及某些进食蚕豆等应激时才氧化性药物，以及某些进食蚕豆等应激时才发生。临床上最严重的并发症是新生儿黄疸。发生。临床上最严重的并发症是新生儿黄疸。GlucoseGlucose- 6-P Fructose-6-PLactatePyruvate kinaseGlucose-6-phosphate dehydrogenaseNAPDNADPH+H+ 2GSHH2OO-GSSGHexo

57、se monophosphate shuntEmbden-Meyerhof glycolytic pathwayRibulose 5-P6-PGOxidant stress!ADPADPATPATPRed cell functioning adequately under normal conditionsDrugs: e.g. antimalarialsFava beansInfectionRed cell cannot produce enough NADPH via the HMP shuntInadequate amounts of GSH to combat oxidant stre

58、ssOxidant damage to cell membraneReduced red cell survivalHaemolytic anaemia!Disorders of Red Cells Resulting from Enzyme Abnormalities3. Cell Metabolism (Disease)Disorders of Red Cells Resulting from Enzyme AbnormalitiesPyruvate kinase deficiency丙酮酸激酶缺陷是引起非球形红细胞溶血性贫血的常见缘由。丙酮酸激酶缺陷是引起非球形红细胞溶血性贫血的常见缘由

59、。3. Cell Metabolism (Disease)3. Cell Metabolismiron metabolism铁分布iron metabolism3. Cell Metabolism铁的出入平衡iron metabolism3. Cell Metabolism人体铁循环铁在一个几乎完全封锁的系统内严密保管。3. Cell Metabolismiron metabolism铁的吸收肠绒毛细胞从肠道摄入铁并转运到血浆DMT-1，二价金属离子运转蛋白Ferritin，铁蛋白Ferroportin，膜铁运转蛋白Hephaestin，膜铁转运辅助蛋白Erythroid bone marro

60、w (normoblasts)Reticuloendothelial system;Spleen & macrophagesDuodenumSerum transferrinFeRed blood cellsLiver2. Iron is then attached to a protein, transferrin in the serum (plasma), where it is transported to the bone marrow for haemoglobin synthesis. 1. Iron is absorbed from the small intestine in the ferrou