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Related Concept Videos

ATP Synthase: Mechanism01:48

ATP Synthase: Mechanism

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In animals, the mitochondrial F1F0 ATP synthase is the key protein that synthesizes ATP molecules through a complex catalytic mechanism. While the nuclear genome encodes the majority of ATP synthase subunits, the mitochondrial genome encodes some of the enzyme's most critical components. The formation of this multi-subunit enzyme is a complex multi-step process regulated at the level of transcription, translation, and assembly. Defects in one or more of these steps can result in decreased...
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ATP Synthase: Structure01:18

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ATP synthase or ATPase is among the most conserved proteins found in bacteria, mammals, and plants. This enzyme can catalyze a forward reaction in response to the electrochemical gradient, producing ATP from ADP and inorganic phosphate. ATP synthase can also work in a reverse direction by hydrolyzing ATP and generating an electrochemical gradient. Different forms of ATP synthases have evolved special features to meet the specific demands of the cell. Based on their specific feature, ATP...
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The Inner Mitochondrial Membrane01:28

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The inner mitochondrial membrane is the primary site of ATP synthesis. The inner membrane domain that forms a smooth layer adjacent to the outer membrane is called the inner boundary membrane. This domain contains membrane transporters that drive metabolites in and out of the mitochondria.  In contrast, the inner membrane network that invaginates into the matrix space is called the cristae membrane. This domain accounts for principle mitochondrial function as it accommodates the protein...
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Chemiosmosis01:32

Chemiosmosis

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Oxidative phosphorylation is a highly efficient process that generates large amounts of adenosine triphosphate (ATP), the basic unit of energy that drives many cellular processes. Oxidative phosphorylation involves two processes— the electron transport chain and chemiosmosis.
Electron Transport Chain
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Mitochondria01:37

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Mitochondria are eukaryotic cellular organelles that are known to produce energy through a process called oxidative phosphorylation. Besides their primary function, mitochondria are involved in various cellular processes, including cell growth, differentiation, signaling, metabolism, and senescence. Age-related changes cause a decline in mitochondrial quality and integrity due to increased mitochondrial mutations and oxidative damage. Thus, aging can severely impact mitochondrial functions,...
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Visualization of ATP Synthase Dimers in Mitochondria by Electron Cryo-tomography
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Understanding structure, function, and mutations in the mitochondrial ATP synthase.

Ting Xu1, Vijayakanth Pagadala2, David M Mueller1

  • 1Department of Biochemistry and Molecular Biology, The Chicago Medical School, Rosalind Franklin University of Medicine and Science, 3333 Green Bay Road, North Chicago, IL 60064.

Microbial Cell (Graz, Austria)
|May 5, 2015
PubMed
Summary
This summary is machine-generated.

Mitochondrial ATP synthase, a molecular motor, synthesizes ATP using proton flow. This review details its structure, function, and how mutations in ATP synthase genes cause human diseases.

Keywords:
ATP synthaseF1 ATPaseF1Fo ATP synthasemitochondrial diseasespetite mutationsuncoupling

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Area of Science:

  • Biochemistry
  • Molecular Biology
  • Genetics

Background:

  • Mitochondrial ATP synthase is a large enzyme complex (approx. 600,000 Da) functioning as a molecular motor.
  • It comprises two main parts: F1 (catalytic sites in mitochondrial matrix) and Fo (proton turbine in inner membrane).
  • Proton flux through Fo powers F1 rotation, driving ATP synthesis, coupling proton flow to energy production.

Purpose of the Study:

  • To review the structure-function relationship of mitochondrial ATP synthase.
  • To link this relationship with disease-causing mutations and SNPs in ATP synthase genes.
  • To understand how alterations in ATP synthase affect enzyme function and individual health.

Main Methods:

  • Biochemical studies
  • Crystallographic analysis
  • Genetic studies

Main Results:

  • The review summarizes subunit composition and roles within ATP synthase.
  • It discusses known mutations and their impact on enzyme activity.
  • It highlights mutations in ATP synthase genes linked to human diseases.

Conclusions:

  • Understanding the structure-function relationship is crucial for deciphering disease mechanisms.
  • Mutations in ATP synthase subunits can lead to severe human pathologies.
  • Further research into SNPs may reveal additional disease associations.