- 同
- SAO
WordNet
- situated in or oriented toward the southeast (同)southeastern, southeasterly
- the compass point midway between south and east; at 135 degrees (同)sou''-east, southeastward, SE
- the direction corresponding to the southeastward compass point
- a location in the southeastern part of a country, region, or city
- to, toward, or in the southeast (同)south-east, sou''-east
- of or relating to or characteristic of Asia or the peoples of Asia or their languages or culture; "Asian countries" (同)Asiatic
- a native or inhabitant of Asia (同)Asiatic
- the largest continent with 60% of the earths population; it is joined to Europe on the west to form Eurasia; it is the site of some of the worlds earliest civilizations
- the nations of the Asian continent collectively
- the southeastern region of the United States (同)southeastern United States
PrepTutorEJDIC
- 〈U〉《the ~》『南東』(《略》『SE,S.E.』) / 《the ~》南東地域,南東部;《the S-》米国南東部 / 南東へ向かう / (風が)南東からの / 南東へ(に)
- 『アジアの』,アジアに住む人々の / 『アジア人』
- 『アジア』
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/02/26 01:43:14」(JST)
[Wiki en表示]
| Southeast Asian ovalocytosis |
| Classification and external resources |
| Specialty |
hematology |
| ICD-10 |
D58.1 |
| ICD-9-CM |
282.1 |
| OMIM |
109270 |
| DiseasesDB |
9416 |
| MeSH |
D004612 |
|
[edit on Wikidata]
|
Southeast Asian ovalocytosis is a form of hereditary elliptocytosis common in some communities in Malaysia and Papua New Guinea, as it confers some resistance to cerebral Falciparum Malaria.[1]
Contents
- 1 Pathophysiology
- 1.1 Southeast Asian ovalocytosis
- 2 See also
- 3 References
Pathophysiology
Southeast Asian ovalocytosis
The primary defect in SAO differs significantly from other forms of elliptocytosis in that it is a defect in the gene coding for a protein that is not directly involved in the cytoskeleton scaffolding of the cell. Rather, the defect lies in a protein known as the band 3 protein, which lies in the cell membrane itself. The band 3 protein normally binds to another membrane-bound protein called ankyrin, but in SAO this bond is stronger than normal. Other abnormalities include tighter tethering of the band 3 protein to the cell membrane, increased tyrosine phosphorylation of the band 3 protein, reduced sulfate anion transport through the cell membrane, and more rapid ATP consumption. These (and probably other) consequences of the SAO mutations lead to the following erythrocyte abnormalities:[2]
- A greater robustness of cells to a variety of external forces, including:
- Reduction in cellular sensitivity to osmotic pressures
- Reduction in fragility related to temperature change
- greater general rigidity of the cell membrane
- Loss of sensitivity to substances that cause spiculation of cells
- Reduced anion exchange
Figure 3 - A representation of the steps involved in the entry of
Plasmodium falciparum into an erythrocyte.
- Partial intracellular depletion of ATP
- A reduction in expression of multiple antigens
These changes are thought to give rise to the scientifically and clinically interesting phenomenon that those with SAO exhibit: a marked in vivo resistance to infection by the causative pathogen of malaria, Plasmodium falciparum. Unlike those with the Leach phenotype of common hereditary elliptocytosis (see above), there is a clinically significant reduction in both disease severity and prevalence of malaria in those with SAO. Because of this, the 35% incidence rate of SAO along the north coast of Madang Province in Papua New Guinea, where malaria in endemic, is a good example of natural selection.[3]
The reasons behind the resistance to malaria become clear when given an explanation the way in which Plasmodium falciparum invades its host. This parasite is an obligate intracellular parasite, which must enter the cells of the host it is invading. The band 3 proteins aggregate on the cell membrane at the site of entry, forming a circular orifice that the parasite squeezes through. These band 3 proteins act as receptors for the parasite. Normally a process much like endocytosis occurs, and the parasite is able to isolate itself from the intracellular proteins that are toxic to it while still being inside an erythrocyte (see figure 2). The increased rigidity of the erythrocyte membrane in SAO is thought to reduce the capacity of the band 3 proteins to cluster together, thereby making it more difficult for the malaria parasite to properly attaching to and enter the cell. The reduced free ATP within the cell has been postulated as a further mechanism behind which SAO creates a hostile environment for Plasmodium falciparum.
See also
- Hereditary elliptocytosis
- Sickle-cell disease
- List of hematologic conditions
References
- ^ "Prevention of cerebral malaria in children in Papua New Guinea by southeast Asian ovalocytosis band 3 -- Allen et al. 60 (6): 1056 -- American Journal of Tropical Medicine and Hygiene". Retrieved 2008-02-26.
- ^ Liu, S.C.; Palek, J; Nichols, PE; Derick, LH; Chiou, SS; Amato, D; Corbett, JD; Golan, DE (1 July 1995). "Molecular basis of altered red blood cell membrane properties in Southeast Asian ovalocytosis: role of the mutant band 3 protein in band 3 oligomerization and retention by the membrane skeleton". Blood 86 (1): 349–58. PMID 7795244.
- ^ Mgone, Cs; Koki, G; Paniu, Mm; Kono, J; Bhatia, Kk; Genton, B; Alexander, Nd; Alpers, Mp (May 1996). "Occurrence of the erythrocyte band 3 (AE1) gene deletion in relation to malaria endemicity in Papua New Guinea.". Transactions of the Royal Society of Tropical Medicine and Hygiene 90 (3): 228–31. doi:10.1016/S0035-9203(96)90223-0. ISSN 0035-9203. PMID 8758056.
‹ The template below (Diseases of RBCs and megakaryocytes) is being considered for deletion. See templates for discussion to help reach a consensus.›
|
Diseases of red blood cells and clotting (D50–69,74, 280–287)
|
|
Red
blood cells |
| ↑ |
|
|
| ↓ |
| Anemia |
| Nutritional |
- Micro-: Iron-deficiency anemia
- Macro-: Megaloblastic anemia
|
|
Hemolytic
(mostly normo-) |
| Hereditary |
- enzymopathy: G6PD
- glycolysis
- hemoglobinopathy: Thalassemia
- Sickle-cell disease/trait
- HPFH
- membrane: Hereditary spherocytosis
- Minkowski–Chauffard syndrome
- Hereditary elliptocytosis
- Southeast Asian ovalocytosis
- Hereditary stomatocytosis
|
|
| Acquired |
- Drug-induced autoimmune
- Drug-induced nonautoimmune
- Hemolytic disease of the newborn
|
|
|
Aplastic
(mostly normo-) |
- Hereditary: Fanconi anemia
- Diamond–Blackfan anemia
- Acquired: PRCA
- Sideroblastic anemia
- Myelophthisic
|
|
| Blood tests |
- MCV
- Normocytic
- Microcytic
- Macrocytic
- MCHC
|
|
|
| Other |
- Methemoglobinemia
- Sulfhemoglobinemia
- Reticulocytopenia
|
|
|
|
Coagulation/
coagulopathy |
| ↑ |
Hyper-
coagulability |
- primary: Antithrombin III deficiency
- Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden
- Prothrombin G20210A
- Sticky platelet syndrome
- acquired:Thrombocytosis
- DIC
- autoimmune
|
|
|
| ↓ |
Hypo-
coagulability |
| Thrombocytopenia |
- Thrombocytopenic purpura: ITP
- TM
- TTP
- Upshaw Schulman syndrome
- Heparin-induced thrombocytopenia
- May–Hegglin anomaly
|
|
| Platelet function |
- adhesion
- aggregation
- Glanzmann's thrombasthenia
- platelet storage pool deficiency
- Hermansky–Pudlak syndrome
- Gray platelet syndrome
|
|
| Clotting factor |
- Hemophilia
- von Willebrand disease
- Hypoprothrombinemia/II
- XIII
- Dysfibrinogenemia
- Congenital afibrinogenemia
|
|
|
|
|
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- Band 3, the human red cell chloride/bicarbonate anion exchanger (AE1, SLC4A1), in a structural context.
- Reithmeier RA1, Casey JR2, Kalli AC3, Sansom MS3, Alguel Y4, Iwata S4.
- Biochimica et biophysica acta.Biochim Biophys Acta.2016 Jul;1858(7 Pt A):1507-32. doi: 10.1016/j.bbamem.2016.03.030. Epub 2016 Apr 6.
- The crystal structure of the dimeric membrane domain of human Band 3(1), the red cell chloride/bicarbonate anion exchanger 1 (AE1, SLC4A1), provides a structural context for over four decades of studies into this historic and important membrane glycoprotein. In this review, we highlight the key stru
- PMID 27058983
- The evolutionary origins of Southeast Asian Ovalocytosis.
- Paquette AM1, Harahap A2, Laosombat V3, Patnode JM1, Satyagraha A2, Sudoyo H2, Thompson MK1, Yusoff NM4, Wilder JA5.
- Infection, genetics and evolution : journal of molecular epidemiology and evolutionary genetics in infectious diseases.Infect Genet Evol.2015 Aug;34:153-9. doi: 10.1016/j.meegid.2015.06.002. Epub 2015 Jun 3.
- Southeast Asian Ovalocytosis (SAO) is a common red blood cell disorder that is maintained as a balanced polymorphism in human populations. In individuals heterozygous for the SAO-causing mutation there are minimal detrimental effects and well-documented protection from severe malaria caused by Plasm
- PMID 26047685
- [Band 3 deficiency as a cause of hereditary spherocytosis].
- Wada H1, Suemori S, Nakanishi H, Sugihara T.
- [Rinshō ketsueki] The Japanese journal of clinical hematology.Rinsho Ketsueki.2015 Jul;56(7):837-45. doi: 10.11406/rinketsu.56.837.
- Band 3 protein accounts for the largest percentage of whole erythrocyte membrane proteins. Abnormalities in this protein are closely associated with pathologies including hereditary spherocytosis (HS), Southeast Asian ovalocytosis and distant renal tubular acidosis. Currently, EMA binding capacity m
- PMID 26251147
Japanese Journal
- Molecular population genetics of SLC4A1 and Southeast Asian Ovalocytosis
- WILDER Jason A,STONE Jonathan A,PRESTON Elizabeth G,FINN Lauren E,RATCLIFFE Hannah L,SUDOYO Herawati
- Journal of human genetics 54(3), 182-187, 2009-03-01
- NAID 10030729755
- Molecular population genetics of SLC4A1 and Southeast Asian ovalocytosis
- Wilder Jason A.,Stone Jonathan A.,Preston Elizabeth G. [他]
- Journal of Human Genetics 54(3), 182-187, 2009-03
- NAID 40016533911
- Ovalocytosis without band 3 gene 27-bp deletion and malaria infection
- KIMURA MASAKO,SOEMANTRI AUGUSTINUS,SISWANTO JO EDI,ISHIDA TAKAFUMI
- Anthropological science : journal of the Anthropological Society of Nippon 114(2), 161-164, 2006-08-01
- NAID 10017650838
Related Links
- Summary Southeast Asian ovalocytosis (SAO) is a rare hereditary red cell membrane defect characterized by the presence of oval-shaped erythrocytes and with most patients being asymptomatic or occasionally manifesting with ...
- Hereditary ovalocytosis is a rare condition passed down through families (inherited). The blood cells are oval-shaped instead of round. It is a form of ... Newborn infants with ovalocytosis may have anemia and jaundice.
★リンクテーブル★
[★]
- 同
- Southeast Asian ovalocytosis
[★]
- 関
- Asia、Chinese、Japanese
[★]
- 関
- Asian