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Related Concept Videos

Primary Production01:06

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The total amount of energy acquired by primary producers in an ecosystem is called gross primary production (GPP). However, of this energy, producers use some for metabolic processes, and some is lost as heat, decreasing the amount of energy available to the next trophic level. The remaining usable amount of energy is called the net primary productivity (NPP). In terrestrial ecosystems, NPP is driven by climate, while light penetration and nutrient availability drive NPP in aquatic ecosystems.
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Vascular plants, which account for over 90% of the Earth’s vegetation, all undergo primary growth—which lengthens roots and shoots. Many land plants, notably woody plants, also undergo secondary growth—which thickens roots and shoots.
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When less is more: primary immunodeficiency with an autoinflammatory kick.

Angeliki Giannelou1, Qing Zhou, Daniel L Kastner

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Next-generation sequencing identified new genetic causes for autoinflammatory disorders. Mutations in CECR1 (adenosine deaminase 2) and phospholipase Cγ₂ disrupt innate immunity regulators, impacting macrophage and lymphocyte function.

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Area of Science:

  • Genetics
  • Immunology
  • Molecular Biology

Background:

  • Next-generation sequencing advances molecular taxonomy in human disease.
  • Autoinflammatory disorders are often linked to germline mutations affecting innate immunity regulators.

Purpose of the Study:

  • To identify novel genetic causes of unexplained autoinflammatory disorders.
  • To elucidate the molecular mechanisms underlying these newly identified diseases.

Main Methods:

  • Whole-exome sequencing of undiagnosed patients.
  • Analysis of genetic mutations and their functional consequences on immune cells.

Main Results:

  • Loss-of-function mutations in cat eye syndrome chromosome region, candidate 1 (CECR1), encoding adenosine deaminase 2, cause a spectrum of vascular and inflammatory conditions by impairing M2 macrophage differentiation.
  • Distinct mutations in phospholipase Cγ₂ lead to autoinflammation and immunodeficiency by affecting signaling in natural killer cells, mast cells, and B lymphocytes.

Conclusions:

  • Next-generation sequencing is powerful for discovering genetic causes of autoinflammation.
  • Deficiencies in key molecular regulators like CECR1 and phospholipase Cγ₂ can lead to autoinflammatory diseases.
  • These genes may play a role in more common inflammatory conditions.