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Related Concept Videos

Diabetic Neuropathy01:22

Diabetic Neuropathy

DefinitionDiabetic neuropathy is nerve damage caused by long-standing diabetes mellitus. It results directly from prolonged high blood sugar levels.PathophysiologyThe pathophysiology of diabetic neuropathy involves both metabolic and vascular disturbances triggered by chronic hyperglycemia.Metabolic injury: Elevated glucose levels activate the polyol pathway within nerve cells, leading to the accumulation of sorbitol and fructose. This increases oxidative stress, disrupts normal nerve...
Mitochondrial Precursor Proteins01:39

Mitochondrial Precursor Proteins

Mitochondrial precursors are partially unfolded or loosely folded polypeptide chains. Newly synthesized precursors are inhibited from spontaneously folding into their native conformation by the cytosolic chaperones, heat shock proteins 70 (Hsp70), and mitochondrial import stimulation factors (MSFs). Precursors bound to MSFs are guided to the TOM70-TOM37 receptors, while precursors bound to Hsp70  chaperones are targetted to TOM20-TOM22 receptor complexes.
Most of the mitochondrial precursors...
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Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation

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Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...
Protein Import into the Peroxisomes01:27

Protein Import into the Peroxisomes

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Related Experiment Video

Updated: May 7, 2026

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry
08:19

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry

Published on: May 5, 2022

Peripheral neuropathy in mitochondrial disorders.

Davide Pareyson1, Giuseppe Piscosquito, Isabella Moroni

  • 1Clinic of Central and Peripheral Degenerative Neuropathies Unit, Department of Clinical Neurosciences, Milan, Italy.

The Lancet. Neurology
|September 21, 2013
PubMed
Summary
This summary is machine-generated.

Peripheral neuropathy is common in mitochondrial disorders due to issues with mitochondrial function and distribution. Early diagnosis is crucial for genetic counseling and managing treatable conditions.

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Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers
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Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers

Published on: September 29, 2017

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Last Updated: May 7, 2026

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry
08:19

Assessing Mitochondrial Function in Sciatic Nerve by High-Resolution Respirometry

Published on: May 5, 2022

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers
10:31

Three-dimensional Imaging and Analysis of Mitochondria within Human Intraepidermal Nerve Fibers

Published on: September 29, 2017

Area of Science:

  • Neuroscience
  • Genetics
  • Cell Biology

Background:

  • Mitochondrial disorders can manifest as peripheral neuropathy, but the reasons for this association are not fully understood.
  • Mitochondrial dysfunction impacts peripheral nerve health, affecting both function and distribution.

Purpose of the Study:

  • To explore the relationship between mitochondrial disorders and peripheral neuropathy.
  • To highlight the significance of mitochondrial dynamics and genetic defects in peripheral nerve pathology.

Main Methods:

  • Review of recent advances in cellular and molecular biology related to mitochondrial function in peripheral nerves.
  • Analysis of genetic mutations affecting mitochondrial dynamics, DNA replication, and respiratory chain complex V.
  • Correlation of specific genetic defects with observed peripheral neuropathy phenotypes.

Main Results:

  • Mutations in mitochondrial dynamics proteins (fusion/fission) are frequently linked to Charcot-Marie-Tooth disease.
  • Peripheral neuropathies present with varied phenotypes in mitochondrial diseases due to replication/maintenance abnormalities or Complex V defects.
  • Growing identification of nuclear genes and phenotypes expands understanding of mitochondrial disorders.

Conclusions:

  • Understanding mitochondrial dysfunction is key to explaining peripheral neuropathy in these disorders.
  • Early diagnosis of mitochondrial disorders is vital for genetic counseling and identifying treatable conditions.
  • Recognizing mitochondrial defects in patients with peripheral neuropathy is of paramount importance for accurate diagnosis and management.