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Related Concept Videos

ATP Synthase: Structure01:18

ATP Synthase: Structure

17.5K
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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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 Driven Pumps II: P-type Pumps01:34

ATP Driven Pumps II: P-type Pumps

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The P-type pumps are a large family of integral membrane transporter ATPases. They are divided into five major types based on substrate specificity, from I to V.
A typical P-type pump has three cytosolic domains: nucleotide-binding (N), phosphorylation (P), and activator (A) domains. These domains are connected to the membrane-spanning helices by short amino acid segments. ATP hydrolysis and covalent phosphoenzyme intermediate formation are crucial parts of the catalytic cycle. At the highly...
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ATP Driven Pumps III: V-type Pumps01:30

ATP Driven Pumps III: V-type Pumps

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V-type pumps are ATP-driven pumps found in the vacuolar membranes of plants, yeast, endosomal and lysosomal membranes of animal cells, plasma membranes of a few specialized eukaryotic cells, and some prokaryotes. They are also known as the V1Vo-ATPase, that couple ATP hydrolysis to transport protons against a concentration gradient.
The peripheral or cytosolic V1 domain with eight subunits is involved in ATP hydrolysis. The integral or transmembrane V0 domain containing at least five subunits...
5.2K
ATP Driven Pumps I: An Overview01:27

ATP Driven Pumps I: An Overview

10.4K
ATP-driven pumps, also known as transport ATPases, are integral membrane proteins. They have binding sites for ATP located on the membrane's cytosolic side and the ion-conducting domain in the transmembrane region. These pumps use the free energy released from ATP hydrolysis to move the solutes across cell membranes against an electrochemical gradient.
There are four main types of ATP-driven pumps - P-type, V-type, F-type, and ABC transporter. All these pumps are of varying complexities and...
10.4K
The ADP/ATP Carrier Protein01:42

The ADP/ATP Carrier Protein

4.6K
ADP/ATP carrier or AAC protein is the most abundant carrier protein in the inner mitochondrial membrane. It transports large quantities of ADP and ATP, equivalent to the average human body weight, every day. Among other transporters, ACC protein is one of the best-studied members of the mitochondrial carrier protein family. The ADP/ATP carrier protein comprises two transmembrane helices connected to a loop and a single alpha-helix on the matrix side. It switches between two conformational...
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Measuring In Vitro ATPase Activity for Enzymatic Characterization
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Torsins: not your typical AAA+ ATPases.

April E Rose1, Rebecca S H Brown1, Christian Schlieker1,2

  • 1a Department of Molecular Biophysics and Biochemistry , Yale University , New Haven , CT , USA and.

Critical Reviews in Biochemistry and Molecular Biology
|November 24, 2015
PubMed
Summary

Torsin ATPases (Torsins) are unique AAA+ proteins in the endoplasmic reticulum. Understanding Torsins is crucial for treating movement disorders like dystonia caused by mutations.

Keywords:
Early-onset torsion dystoniaLAP1LULL1endoplasmic reticulumnuclear envelopetorsinA

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A Semi-High-Throughput Adaptation of the NADH-Coupled ATPase Assay for Screening Small Molecule Inhibitors
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A Semi-High-Throughput Adaptation of the NADH-Coupled ATPase Assay for Screening Small Molecule Inhibitors
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Area of Science:

  • Biochemistry
  • Cell Biology
  • Molecular Biology

Background:

  • Torsin ATPases (Torsins) are part of the AAA+ family but possess distinct characteristics.
  • They are uniquely localized to the endoplasmic reticulum and perinuclear space.
  • Torsins play essential roles in metazoans, though their functions are not fully understood.

Purpose of the Study:

  • To provide a comprehensive overview of the Torsin system in metazoans.
  • To analyze Torsin structure, phylogeny, and cofactor interactions in relation to disease mutations.
  • To review recent advancements in understanding Torsin molecular mechanisms and their link to movement disorders.

Main Methods:

  • Phylogenetic and structural analysis of Torsins and cofactors.
  • Review of functional data from various model systems.
  • Analysis of disease-associated mutations in Torsins.

Main Results:

  • Torsins exhibit atypical features compared to other AAA+ ATPases.
  • Mutations in Torsins are linked to congenital human disorders, notably early-onset torsion dystonia (DYT1).
  • Recent research has significantly advanced the molecular understanding of Torsin function.

Conclusions:

  • A deeper understanding of the Torsin system is vital for elucidating the molecular basis of dystonia.
  • This knowledge can inform the development of therapeutic strategies for Torsin-related disorders.
  • Further research is needed to fully define the cellular roles of these atypical molecular machines.