默本概念在地中海贫血和呼吸系统疾病中的相关性是什么?

IF 0.6 Q4 HEMATOLOGY
Kelath Murali Manoj
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引用次数: 0

摘要

Murburn概念是理解细胞功能的一个新视角,认为细胞是简单的化学发动机(SCE),由有效电荷分离(ECS)引发的氧化还原反应提供动力。参与这些过程的1电子活性扩散反应(氧)物质(DR(O)S)平衡对于稳态、相干网络细胞和呈现感知和响应刺激的机电功能也至关重要。这一视角呈现了氧气真正的生理功能,即实现ECS和DR(O)S的生成。因此,DR(O)S现在必须被视为生命的灵丹妙药,尽管在错误的数量、地点和时间出现时,它们可能会产生意想不到的效果(即传统上认为的氧化应激)。我们还阐述了四聚体血红蛋白(Hb)实际上是一种atp合成murburn酶(一种通过murburn概念起作用的酶),并假设murburn活性可能导致Hb上的几种翻译后修饰(如糖化)。Murburn的观点也建立了一个简单的理论基础来解释红细胞在3-4个月的维持,尽管它们缺乏细胞核或线粒体(分别协调它们的各种功能和大量产生ATP)。尽管地中海贫血的根源在于遗传原因,但对氧-血红蛋白-红细胞三重奏机制作用的新认识显著影响了我们解释研究数据和设计治疗这种疾病的方法。这些见解也适用于涉及呼吸窘迫(如哮喘、肺癌、COVID-19和肺炎)和线粒体疾病的其他临床表现。在此,简要讨论这些背景和发展。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
What Is the Relevance of Murburn Concept in Thalassemia and Respiratory Diseases?
Murburn concept is a novel perspective for understanding cellular function, deeming cells as simple chemical engines (SCE) that are powered by redox reactions initiated by effective charge separation (ECS). The 1-electron active diffusible reactive (oxygen) species, or DR(O)S, equilibriums involved in these processes are also crucial for homeostasis, coherently networking cells, and rendering electromechanical functions of sensing and responding to stimuli. This perspective presents the true physiological function of oxygen, which is to enable ECS and the generation of DR(O)S. Therefore, DR(O)S must now to be seen as the quintessential elixir of life, although they might have undesired effects (i.e., the traditionally perceived oxidative stress) when present in the wrong amounts, places and times. We also elaborated that tetrameric hemoglobin (Hb) is actually an ATP-synthesizing murzyme (an enzyme working via murburn concept) and postulated that several post-translational modifications (such as glycation) on Hb could result from murburn activity. Murburn perspective has also enabled the establishment of a facile rationale explaining the sustenance of erythrocytes for 3–4 months, despite their lacking nucleus or mitochondria (to coordinate their various functions and mass-produce ATP, respectively). Although thalassemia has its roots in genetic causation, the new awareness of the mechanistic roles of oxygen-hemoglobin-erythrocyte trio significantly impacts our approaches to interpreting research data and devising therapies for this malady. These insights are also relevant in other clinical manifestations that involve respiratory distress (such as asthma, lung cancer, COVID-19 and pneumonia) and mitochondrial diseases. Herein, these contexts and developments are briefly discussed.
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来源期刊
Thalassemia Reports
Thalassemia Reports HEMATOLOGY-
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