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Related Concept Videos

Centrosome Duplication02:25

Centrosome Duplication

The primary microtubule organizing center (MTOC) in animal cells is the centrosome. A centrosome has two cylindrical centrioles at its core. Each centriole consists of nine sets of three microtubules held together by proteins. The centrioles are positioned at right angles to each other and surrounded by a shapeless protein cloud called the pericentriolar matrix, or pericentriolar material (PCM).
To ensure that each daughter cell receives a centrosome after cell division, centrosome duplication...
Centrosome Duplication02:25

Centrosome Duplication

The primary microtubule organizing center (MTOC) in animal cells is the centrosome. A centrosome has two cylindrical centrioles at its core. Each centriole consists of nine sets of three microtubules held together by proteins. The centrioles are positioned at right angles to each other and surrounded by a shapeless protein cloud called the pericentriolar matrix, or pericentriolar material (PCM).
To ensure that each daughter cell receives a centrosome after cell division, centrosome duplication...
Centrioles and Centrosomes01:13

Centrioles and Centrosomes

Most animal cells comprise a pair of centrioles together called a centrosome. The cell duplicates its centrosome and contains two centrosomes side-by-side, which begin to move apart during the prophase. As the centrosomes migrate to two different sides of the cell, microtubules start extending from each centrosome toward the other end. The mitotic spindle is composed of the centrosomes and their emerging microtubules.
Near the end of the prophase, also called late prophase or "prometaphase,"...
Microtubule Formation01:23

Microtubule Formation

Microtubules are dynamic structures that undergo continuous assembly and disassembly. They originate from specialized multi-protein complexes known as microtubule organizing centers or MTOCs. Within the MTOC, the point of origin of the microtubule is known as the minus end, while the end radiating outward is the plus end. Microtubules serve two primary functions — the organization of spindle complexes to separate sister chromatids during mitotic or meiotic cell division and the formation of...
Histone Variants at the Centromere02:30

Histone Variants at the Centromere

Histone variants are the histone proteins with structural and sequence variations. These variants may be regarded as “mutant” forms that replace their canonical histone counterparts in the nucleosomes. Specific post-translational modifications on the histone variants enable further chromatin complexity and regulate tissue-specific gene expression. The most common histone variants are from histone H2A, H2B, and linker histone H1 families. However, several variants of histone H3 variants are also...
Spindle Assembly02:50

Spindle Assembly

Spindle assembly occurs through three, often coexisting, pathways – the centrosome-mediated pathway, the chromatin-mediated pathway, and the microtubule-mediated pathway – collectively contributing to form a robust spindle apparatus.
In most cells, centrosomes are the primary microtubule nucleation centers. In the centrosome-mediated pathway, the G2-prophase transition triggers centrosome maturation and increased microtubule nucleation. Progressive nucleation results in a microtubule array...

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Related Experiment Video

Updated: May 28, 2026

Quantitative Immunofluorescence Assay to Measure the Variation in Protein Levels at Centrosomes
09:39

Quantitative Immunofluorescence Assay to Measure the Variation in Protein Levels at Centrosomes

Published on: December 20, 2014

A primary microcephaly protein complex forms a ring around parental centrioles.

Joo-Hee Sir1, Alexis R Barr, Adeline K Nicholas

  • 1Cancer Research UK Cambridge Research Institute, Li Ka Shing Centre, Cambridge, UK.

Nature Genetics
|October 11, 2011
PubMed
Summary
This summary is machine-generated.

Primary microcephaly (MCPH) is linked to centrosome protein defects. A mutation in CEP63 disrupts a CEP152-CEP63 ring structure essential for normal brain development.

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Related Experiment Videos

Last Updated: May 28, 2026

Quantitative Immunofluorescence Assay to Measure the Variation in Protein Levels at Centrosomes
09:39

Quantitative Immunofluorescence Assay to Measure the Variation in Protein Levels at Centrosomes

Published on: December 20, 2014

Immunofluorescence Analysis of Endogenous and Exogenous Centromere-kinetochore Proteins
05:35

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Published on: March 3, 2016

Identification of Cyclin-dependent Kinase 1 Specific Phosphorylation Sites by an In Vitro Kinase Assay
12:26

Identification of Cyclin-dependent Kinase 1 Specific Phosphorylation Sites by an In Vitro Kinase Assay

Published on: May 3, 2018

Area of Science:

  • Cell Biology
  • Neuroscience
  • Genetics

Background:

  • Autosomal recessive primary microcephaly (MCPH) is a neurodevelopmental disorder characterized by reduced brain size.
  • MCPH arises from mutations in genes encoding centrosomal proteins, but the precise centrosome dysfunction remains unclear.
  • Centrosomes are critical for cell division and organization, and their proper function is vital for brain development.

Purpose of the Study:

  • To investigate the role of the CEP63 gene in autosomal recessive primary microcephaly (MCPH).
  • To elucidate the function of CEP63 and its interaction with other centrosomal proteins in maintaining centrosome number and integrity.
  • To understand how CEP63 dysfunction impacts human brain development, particularly cerebral cortex growth.

Main Methods:

  • Genetic analysis to identify mutations in CEP63 in MCPH patients.
  • Biochemical assays to study the interaction between CEP63 and CEP152.
  • Super-resolution microscopy to visualize the localization and structure of CEP63 and CEP152 at the centrosome.

Main Results:

  • A homozygous MCPH-causing mutation was identified in the human CEP63 gene.
  • CEP63 forms a complex with CEP152, and together they are essential for maintaining normal centrosome numbers.
  • CEP63 and CEP152 form a ring structure at the proximal end of the parental centriole, which is disrupted in CEP63-deficient cells.

Conclusions:

  • The CEP152-CEP63 ring structure is crucial for normal neurodevelopment.
  • Impairment of this ring structure, particularly due to CEP63 mutations, significantly affects human cerebral cortex growth.
  • This study provides insights into the molecular mechanisms underlying MCPH and highlights the importance of centrosome integrity in brain development.