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ATP Synthase: Structure01:18

ATP Synthase: Structure

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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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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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Functional groups are a group of atoms with characteristic properties, which when linked to the carbon skeleton of a molecule, alter the properties of that molecule. For example, the presence of certain functional groups on a molecule will make them hydrophilic, whereas others will make them hydrophobic. These functional groups are an indispensable part of organic chemistry and important components of biological molecules, such as carbohydrates, proteins, lipids, and nucleic acids. Each...
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Visual System01:26

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Light enters the eye through the cornea, a transparent, dome-shaped surface covering the surface of the eyeball that helps to direct and focus incoming light. This light is then channeled toward the pupil, an adjustable opening whose size is controlled by the iris. The iris, a pigmented muscle, regulates the amount of light entering the eye by contracting or dilating the pupil, thereby ensuring optimal light levels for clear vision.
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Functionalism01:11

Functionalism

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William James, John Dewey, and Charles Sanders Peirce were instrumental in founding functional psychology, which draws heavily from Darwin's theory of evolution by natural selection. This theory suggests that individual traits, including behaviors, are adapted to their environments through natural selection. At the heart of functionalism is the concept of adaptation, meaning that a trait enhances an individual's chances of survival and reproduction.
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Visualization of ATP Synthase Dimers in Mitochondria by Electron Cryo-tomography
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Visual Function in Mice Lacking GM3 Synthase.

Miki Hiraoka1, Ei Ohkawa2, Akira Abe1

  • 1a Department of Ophthalmology , School of Medicine, Sapporo Medical University , Sapporo , Japan.

Current Eye Research
|January 29, 2019
PubMed
Summary

GM3 synthase deficiency in mice did not cause visual impairment or retinal abnormalities, unlike in humans. These findings suggest GM3

Keywords:
GM3Gangliosidesanti-glial fibrillary acidic protein (GFAP)electroretinogram (ERG)micevisual evoked potential (VEP)

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Area of Science:

  • Neuroscience
  • Biochemistry
  • Genetics

Background:

  • Gangliosides, like GM3, are crucial complex lipids in vertebrates.
  • GM3 deficiency in humans is linked to epilepsy and visual impairment.
  • The ST3GAL5 gene encodes GM3 synthase, essential for GM3 production.

Purpose of the Study:

  • To investigate the role of GM3 deficiency in visual function using ST3GAL5 knockout mice.
  • To determine if GM3 synthase mutations impact retinal morphology and function.

Main Methods:

  • Analysis of retinal glycosphingolipids in ST3GAL5-/- mice using high-performance thin-layer chromatography.
  • Histopathological evaluation of retinas and optic nerves via H&E staining and GFAP immunohistochemistry.
  • Functional assessment using electroretinography (ERG) and visual evoked potential (VEP) at various ages.

Main Results:

  • GM3 was absent in ST3GAL5-/- mouse retinas, with compensatory increases in GM1b, GD1α, and lactosylceramide.
  • No significant differences in GFAP expression were observed between wild-type and knockout mice.
  • ERG and VEP tests revealed no functional visual deficits in GM3-deficient mice.

Conclusions:

  • GM3 deficiency in mice does not result in histopathological or functional abnormalities of the retina or optic nerve.
  • Contrary to human conditions, lack of GM3 in mice does not lead to optic nerve atrophy.
  • These results highlight species-specific differences in the role of GM3 in visual system integrity.