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Related Concept Videos

Conformity01:20

Conformity

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Conformity is the change in a person’s behavior to go along with the group, even if that person does not agree with the group.
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Conformations of Butane

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Unlike ethane and propane that have only two major conformations, butane has more than two conformers. The staggered form of butane in which the bulky methyl groups on the two carbons are placed on opposite sides, that is, at a dihedral angle of 180°, is the lowest energy, most stable form — called the anti conformer. This conformation is stabilized due to the absence of steric repulsion between the largely spaced out methyl groups. The other two staggered conformations are...
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Conformations of Cycloalkanes02:29

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Adolf von Baeyer attempted to explain the instabilities of small and large cycloalkane rings using the concept of angle strain — the strain caused by the deviation of bond angles from the ideal 109.5° tetrahedral value for sp3  hybridized carbons. However, while cyclopropane and cyclobutane are strained, as expected from their highly compressed bond angles, cyclopentane is more strained than predicted, and cyclohexane is virtually strain-free. Hence, Baeyer’s theory that...
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Cyclohexane does not exist in a planar form due to the high angle and torsional strain it would experience in the planar structure. Instead, it adopts non-planar chair and boat conformations.
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Single Nucleotide Polymorphisms-SNPs01:05

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A single nucleotide polymorphism or SNP is a single nucleotide variation at a specific genomic position in a large population. It is the most prevalent type of sequence variation found in the human genome. Point mutations that occur in more than 1% of the population qualify as SNPs. These are present once every 1000 nucleotides on an average in the human genome. Replacement of a purine with another purine (A/G) or a pyrimidine with another pyrimidine (C/T) is known as a transition. In contrast,...
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Conformations of Ethane and Propane02:18

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In an organic molecule, free rotation about the carbon-carbon single bond results in energetically different conformers of the molecule. Due to this rotation, called the internal rotation, ethane has two major conformations — staggered and eclipsed.
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Conformation Polymorphism of Polyglutamine Proteins.

Xinran Feng1, Shouqing Luo2, Boxun Lu1

  • 1Neurology Department at Huashan Hospital, State Key Laboratory of Medical Neurobiology, School of Life Sciences, Fudan University, Shanghai, China.

Trends in Biochemical Sciences
|April 12, 2018
PubMed
Summary

Expanded polyglutamine (polyQ) protein structures are key to understanding neurodegenerative diseases. This review examines hypotheses on polyQ structure and toxicity, focusing on Huntingtin protein, and proposes new research strategies.

Keywords:
antibodyautophagyneurodegenerationpolyQprotein misfolding

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Area of Science:

  • Biochemistry and Molecular Biology
  • Neuroscience
  • Structural Biology

Background:

  • Expanded polyglutamine (polyQ) stretches in proteins cause at least nine human diseases.
  • The structural basis of polyQ-induced pathogenesis remains unclear due to a lack of atomic-level structural data.
  • Understanding the structure-cytotoxicity relationship is crucial for polyQ disease mechanisms.

Purpose of the Study:

  • To review existing hypotheses on the structure-cytotoxicity relationship of pathogenic polyQ proteins.
  • To integrate insights from recent structural and chemical biology studies.
  • To propose novel strategies for clarifying the polyQ protein conformation-cytotoxicity link.

Main Methods:

  • Literature review of hypotheses and supporting evidence.
  • Analysis of recent structural biology findings.
  • Integration of chemical biology insights.
  • Focus on Huntingtin (HTT) protein structure and function.

Main Results:

  • Multiple hypotheses exist regarding polyQ structure and cytotoxicity, often based on indirect evidence.
  • Recent structural and chemical biology studies offer new perspectives.
  • Huntingtin (HTT) is the most studied polyQ disease protein, providing a key model.

Conclusions:

  • Clarifying the precise structure of polyQ proteins is essential for understanding disease pathogenesis.
  • Further research integrating structural and chemical biology is needed.
  • Novel strategies may elucidate the conformation-cytotoxicity relationship in polyQ diseases.