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Related Concept Videos

Peroxisomes01:24

Peroxisomes

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Peroxisomes are specialized organelles present in fungi, plant, and animal cells. It can vary in number, size, morphology, and activity depending on the type of tissue and the nutritional state of the cell. For example, cells with active lipid metabolism, such as adipocytes, neurons, and hepatocytes, have more peroxisomes than other cells in the body. Besides their primary role in breaking down complex organic molecules, peroxisomes can also synthesize specific macromolecules and participate in...
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Protein Import into the Peroxisomes01:27

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Cells contain membrane-bound organelles called peroxisomes that oxidize organic molecules by transferring hydrogen atoms to oxygen, producing hydrogen peroxide. Peroxisomes enzymatically convert the released hydrogen peroxide into water and oxygen.
Peroxisomal Protein Import:
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Peroxisomes and Mitochondria01:30

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Peroxisomes and mitochondria are two important oxygen-utilizing organelles in eukaryotic cells. Mitochondria carry out cellular respiration—the process that converts energy from food into ATP. Peroxisomes carry out a variety of functions, primarily breaking down different substances, such as fatty acids.
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Lipid Catabolism01:25

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Triglycerides serve as crucial long-term energy storage molecules in microorganisms, providing a dense source of metabolic energy. Their breakdown is mediated by lipases, which hydrolyze triglycerides into glycerol and free fatty acids. Each of these components follows distinct metabolic pathways, ultimately contributing to ATP synthesis and cellular energy homeostasis.Glycerol MetabolismGlycerol, released from triglyceride hydrolysis, is phosphorylated by glycerol kinase to form...
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Porin Insertion in the Outer Mitochondrial Membrane01:12

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Porins are beta-barrel proteins translocated to the mitochondrial outer membrane through the TOM complex into the intermembrane space. Porin precursors bind TIM chaperones within the intermembrane space and are guided to the Sorting and Assembly Machinery complex or SAM complex on the outer mitochondrial membrane.
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Regulated Protein Degradation02:58

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It is vital to regulate the activity of enzymatic as well as non-enzymatic proteins inside the cell. This can be achieved either through creating a balance between their rate of synthesis and degradation or regulating the intrinsic activity of the protein. Both these regulation mechanisms play an essential role in the normal functioning of cells.
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VPS13D promotes peroxisome biogenesis.

Heather A Baldwin1,2, Chunxin Wang1, Gil Kanfer1

  • 1Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.

The Journal of Cell Biology
|April 23, 2021
PubMed
Summary

The VPS13D gene is crucial for maintaining peroxisome function. Loss of VPS13D disrupts peroxisome biogenesis, impacting cell health and potentially causing diseases like spinocerebellar ataxia.

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Area of Science:

  • Cell Biology
  • Genetics
  • Human Disease

Background:

  • The VPS13 gene family, including VPS13A-D, is implicated in human diseases.
  • The specific cellular roles of VPS13D remain largely unknown.
  • Understanding VPS13D function is critical for disease research.

Purpose of the Study:

  • To investigate the cellular functions of the VPS13 gene family, with a focus on VPS13D.
  • To characterize the impact of VPS13 gene knockouts on cellular morphology and organelle integrity.
  • To determine the role of VPS13D in peroxisome biogenesis and its link to human pathologies.

Main Methods:

  • Generation and characterization of individual VPS13 gene knockouts in HeLa cells.
  • Microscopic analysis of mitochondrial and peroxisome morphology in knockout cell lines.
  • Investigation of VPS13D function in patient-derived fibroblasts with spinocerebellar ataxia.

Main Results:

  • VPS13D knockout cells displayed abnormal mitochondrial morphology.
  • Loss of VPS13D resulted in partial or complete peroxisome loss across multiple cell types.
  • Fibroblasts from a VPS13D mutation patient showed peroxisome deficiency.

Conclusions:

  • VPS13D plays a vital role in regulating peroxisome biogenesis.
  • VPS13D deficiency leads to peroxisome loss and associated cellular defects.
  • Further research into VPS13D is warranted for understanding spinocerebellar ataxia and related disorders.