23.Leukocyte adhesion deficiency-I 起因於下列何種蛋白質的基因缺陷?
(A)Neutrophil elastase
(B)Lamin B receptor
(C)β2-integrin
(D)NADPH oxidase
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統計: A(329), B(212), C(1028), D(164), E(0) #3197961
統計: A(329), B(212), C(1028), D(164), E(0) #3197961
詳解 (共 6 筆)
#6132842
Leukocyte adhesion deficiency:
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Neutrophil的β2-integrin(CD11 CD18/LFA-1) & ICAM-1連接異常:
LAD-I: CD18異常
LAD-III:β2-integrin activation異常
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LAD-II:Neutrophil的E-selecin & CD15(Slalyted Lewisx)連接有異常
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#7272404
Leukocyte adhesion deficiency:
LAD-I: CD18異常
LAD-II:Neutrophil的E-selecin & CD15(Slalyted Lewisx)連接有異常
LAD-III:β2-integrin activation異常
LAD-III:β2-integrin activation異常
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#6055321
" β2 integrins are heterodimeric surface receptors composed of a variable α (CD11a-CD11d) and a constant β (CD18) subunit and are specifically expressed by leukocytes. The α subunit defines the individual functional properties of the corresponding β2 integrin, but all β2 integrins show functional overlap. They mediate adhesion to other cells and to components of the extracellular matrix (ECM), orchestrate uptake of extracellular material like complement-opsonized pathogens, control cytoskeletal organization, and modulate cell signaling. "
" LAD-I (leukocyte adhesion deficiency 1) patients suffer from strong recurrent infections. These immune defects have been largely attributed to impaired migratory and phagocytic properties of polymorphonuclear granulocytes. The molecular base for this inherited disease is a functional impairment of β2 integrins due to mutations within the CD18 gene. β2 integrin activity on leukocytes has been implicated in tumor development."
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Monika Bednarczyk, Henner Stege, Stephan Grabbe, and Matthias Bros (2020). β2 Integrins—Multi-Functional Leukocyte Receptors in Health and Disease. Int J Mol Sci. 2020 Feb; 21(4): 1402. doi: 10.3390/ijms21041402.
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