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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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A single mitochondrion is a bean-shaped organelle enclosed by a double-membrane system. The outer membrane of mitochondria is smooth and contains many porins - the integral membrane transporters. Porins enable free diffusion of ions and small uncharged molecules through the outer mitochondrial membrane but limit the transport of molecules larger than 5000 Daltons. Further, the outer mitochondrial membrane forms a unique structure called membrane contact sites with other subcellular organelles,...
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Mitochondria are double-membrane organelles of the eukaryotes involved in cellular metabolism, signaling, ATP synthesis, and programmed cell death.  Each of these processes requires specific proteins and enzymes that must be correctly sorted to the right mitochondrial subcompartment for the proper functioning of the organelle.
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Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry
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Mitochondrial function requires NGLY1.

Jianping Kong1, Min Peng1, Julian Ostrovsky1

  • 1Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

Mitochondrion
|July 29, 2017
PubMed
Summary
This summary is machine-generated.

Cellular deglycosylation is crucial for mitochondrial function. NGLY1 deficiency impairs mitochondrial respiration, impacting diseases like congenital disorders of glycosylation (CDG) and respiratory chain disorders.

Keywords:
C. ElegansFibroblastsGlycosylationMitochondriaN-glycanase

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Area of Science:

  • Biochemistry
  • Cell Biology
  • Genetics

Background:

  • Mitochondrial respiratory chain (RC) diseases and congenital disorders of glycosylation (CDG) exhibit clinical overlap but distinct pathophysiology.
  • NGLY1 deficiency is a CDG subtype impacting cellular deglycosylation.

Purpose of the Study:

  • To investigate the link between N-linked deglycosylation and mitochondrial function.
  • To evaluate mitochondrial physiology in models of NGLY1 deficiency.

Main Methods:

  • Analysis of mitochondrial amount and function in patients and NGLY1-deficient cell/organism models (fibroblasts, mouse embryonic fibroblasts, C. elegans).
  • Assessment of mitochondrial membrane potential, oxidant burden, and respiratory capacity.
  • Lentiviral rescue experiments to confirm NGLY1's role.

Main Results:

  • NGLY1 deficiency consistently disrupted mitochondrial physiology across models.
  • Observed were reduced mitochondrial content, impaired membrane potential, increased oxidant burden, and decreased respiratory capacity.
  • Lentiviral rescue restored NGLY1 expression and mitochondrial function.

Conclusions:

  • Cellular deglycosylation capacity is a significant factor in mitochondrial RC disease pathogenesis.
  • NGLY1 directly influences mitochondrial function, highlighting a shared pathway.
  • This finding bridges the understanding between CDG and mitochondrial disorders.