Related Experiment Video
Updated: Jun 27, 2026
08:19
A Pipeline to Characterize Structural Heart Defects in the Fetal Mouse
Published on: December 16, 2022
COG defects, birth and rise!
1Unité de Glycobiologie Structurale et Fonctionnelle UMR/CNRS 8576, IFR147, Université des Sciences et Technologies de Lille, France. francois.foulquier@univ-lille1.fr
Biochimica Et Biophysica Acta
|November 26, 2008
Summary
The Golgi COG complex is crucial for retrograde Golgi trafficking and glycosylation. Deficiencies in COG subunits cause Congenital Disorders of Glycosylation (CDG), a group of inherited glycosylation defects.
Area of Science:
- Cell Biology
- Genetics
- Biochemistry
Background:
- The COG complex is an 8-subunit cytosolic heteromeric complex essential for retrograde vesicular transport within the Golgi apparatus.
- COG complex function is critical for the localization of Golgi glycosyltransferases, impacting protein glycosylation.
- Deficiencies in COG subunits are linked to Congenital Disorders of Glycosylation (CDG), a class of inherited glycosylation defects.
Purpose of the Study:
- To review the emergence and progression of COG subunit deficiencies as a cause of CDG.
- To discuss the structural architecture of the COG complex.
- To elucidate the cellular functions of the COG complex in Golgi trafficking and glycosylation.
Main Methods:
- Literature review of COG complex research.
- Analysis of genetic data from CDG patients.
- Functional studies on COG complex subunits and their role in Golgi trafficking.
Main Results:
- Numerous COG-deficient CDG patients have been identified, highlighting the clinical significance of this group of disorders.
- The COG complex's role in Golgi glycosylation and the localization of key enzymes has been established.
- Understanding COG complex architecture provides insights into its function in retrograde transport.
Conclusions:
- COG complex dysfunction is a significant cause of Congenital Disorders of Glycosylation.
- Further research into COG complex structure and function is vital for understanding and potentially treating CDG.
- This review consolidates current knowledge on COG-related CDG, charting the rise of these genetic defects.
Related Concept Videos
01:23Lumber Defects
Lumber defects, which can affect both the appearance and structural integrity of wood, include a variety of growth and manufacturing flaws. Growth defects such as knots and knotholes occur where branches were once attached to the tree trunk, with knotholes forming when these knots fall out. Other natural defects include decay and insect damage, which compromise the wood's strength and durability.
Shakes are minor fractures that run along or across the wood's annual rings, while wane is...
Shakes are minor fractures that run along or across the wood's annual rings, while wane is...
01:14Fetal Circulation
Fetal circulation is a unique system that facilitates the exchange of gases, nutrients, and waste products between the developing fetus and the mother. This intricate process takes place through a special organ called the placenta.
Two umbilical arteries transport blood from the fetus to the placenta. At the placenta, the blood absorbs oxygen and nutrients while simultaneously eliminating waste products. This oxygen-enriched and nutrient-rich blood then returns to the fetus through one...
Two umbilical arteries transport blood from the fetus to the placenta. At the placenta, the blood absorbs oxygen and nutrients while simultaneously eliminating waste products. This oxygen-enriched and nutrient-rich blood then returns to the fetus through one...
01:07Teratogenicity
The ability of a drug to produce structural deformations and functional abnormalities in the developing embryo or the fetus is called teratogenicity, and the drug producing this effect is known as a teratogen. Teratogenic effects include stillbirth, miscarriage, intrauterine growth restriction, and neurocognitive delay. A teratogen may affect the embryo at different stages of development, which is important in determining the type and extent of the damage. During blastocyst formation, the early...
01:29Nondisjunction
During meiosis, chromosomes occasionally separate improperly. This occurs due to failure of homologous chromosome separation during meiosis I or failed sister chromatid separation during meiosis II. In some species, notably plants, nondisjunction can result in an organism with an entire additional set of chromosomes, which is called polyploidy. In humans, nondisjunction can occur during male or female gametogenesis and the resulting gametes possess one too many or one too few chromosomes.
01:29Nondisjunction
During meiosis, chromosomes occasionally separate improperly. This occurs due to failure of homologous chromosome separation during meiosis I or failed sister chromatid separation during meiosis II. In some species, notably plants, nondisjunction can result in an organism with an entire additional set of chromosomes, which is called polyploidy. In humans, nondisjunction can occur during male or female gametogenesis and the resulting gametes possess one too many or one too few chromosomes.
01:21Nondisjunction
Nondisjunction is the failure of homologous chromosomes or sister chromatids to separate correctly and move to the opposite poles of the cells. This produces daughter cells with abnormal chromosome numbers. Nondisjunction is common during anaphase I or anaphase II of meiosis. Mutations in synaptonemal complex proteins that attach homologous chromosomes increase the chances of nondisjunction in anaphase I of meiosis I. In contrast, mutations in topoisomerases and condensins that hold sister...