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Related Concept Videos

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...
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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,...
Mitochondrial Membranes01:45

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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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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 ATP...
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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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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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Histological Examination of Mitochondrial Morphology in a Parkinson's Disease Model
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Abnormal growth in mitochondrial disease.

S Wolny1, R McFarland, P Chinnery

  • 1Department of Paediatrics, Royal Victoria Infirmary, Newcastle Upon Tyne, UK. Susanne.Wolny@nuth.nhs.uk

Acta Paediatrica (Oslo, Norway : 1992)
|January 6, 2009
PubMed
Summary

Children with mitochondrial disease often experience short stature and declining BMI. Further research is needed to understand the underlying causes of abnormal growth in pediatric mitochondrial disease.

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Area of Science:

  • Pediatric Endocrinology
  • Mitochondrial Diseases
  • Growth and Development

Background:

  • Mitochondrial diseases are a group of inherited metabolic disorders affecting cellular energy production.
  • Abnormal growth patterns, including short stature and low body weight, are frequently observed in children with these conditions.
  • Understanding growth parameters is crucial for managing the overall health and well-being of affected children.

Purpose of the Study:

  • To assess and review the height and weight status of pediatric patients diagnosed with mitochondrial disease.
  • To analyze growth parameters within a supra-regional service context.

Main Methods:

  • Retrospective audit of cross-sectional data.
  • Included 24 children and adolescents (median age 7.86 years) with confirmed mitochondrial disease.
  • Height and weight measurements were converted to Standard Deviation (SD) scores using UK reference data.

Main Results:

  • Patients exhibited short stature, with an overall mean SD score of -1.97.
  • A relatively low Body Mass Index (BMI) SD score of -1.07 was observed.
  • BMI SD scores showed a significant negative correlation with advancing age (r = -0.7; p < 0.000).

Conclusions:

  • Short stature and a progressive decrease in BMI are characteristic features of mitochondrial disease in childhood.
  • These findings highlight the significant impact of mitochondrial disease on pediatric growth.
  • Further investigation into the mechanisms driving abnormal growth in these patients is warranted.