Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Fibrillin microfibrils.

Cay M Kielty1, Michael J Sherratt, Andrew Marson

  • 1Wellcome Trust Centre for Cell-Matrix Research, Faculty of Life Sciences, University of Manchester, Manchester M13 9PT, United Kingdom.

Advances in Protein Chemistry
|April 20, 2005
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Spontaneous spinal CSF leaks: a rare variant exome sequencing study and functional analysis.

The Lancet. Neurology·2026
Same author

Structural and biophysical analysis of the four CHRD domains of human chordin reveals a novel binding site for glycosaminoglycans.

The Journal of biological chemistry·2026
Same author

Comparative circadian transcriptome analysis reveals dampened and phase-advanced rhythms in sun-exposed human skin.

The Journal of investigative dermatology·2026
Same author

Bioactive peptide matrikines: discovery approaches for skin rejuvenation.

American journal of physiology. Cell physiology·2026
Same author

Human pluripotent stem cell model of multiple epiphyseal dysplasia with MATN3 mutation identifies altered matrix organisation and upregulation of the cholesterol biosynthesis pathway.

Osteoarthritis and cartilage·2026
Same author

An avidity-driven mechanism of extracellular BMP regulation by Twisted gastrulation.

Development (Cambridge, England)·2026
Same journal

The plasma proteins and their fractionation.

Advances in protein chemistry·2010
Same journal

The preparation and criteria of purity of the amino acids.

Advances in protein chemistry·2010
Same journal

The use of protein and protein hydrolyzates for intravenous alimentation.

Advances in protein chemistry·2010
Same journal

The amino acid requirements of man.

Advances in protein chemistry·2010
Same journal

Reactions of native proteins with chemical reagents.

Advances in protein chemistry·2010
Same journal

The chemical determination of proteins.

Advances in protein chemistry·2010
See all related articles

Fibrillin microfibrils provide elasticity to connective tissues and are crucial for elastic fiber formation. Understanding their assembly and properties is key to addressing Marfan syndrome, a genetic disorder caused by fibrillin-1 mutations.

Area of Science:

  • Biochemistry
  • Cell Biology
  • Connective Tissue Research

Background:

  • Fibrillin microfibrils are essential extracellular matrix components contributing to tissue elasticity.
  • They play roles in elastic fiber formation and are found in both elastic and non-elastic tissues.
  • Mutations in fibrillin-1 cause Marfan syndrome, highlighting the clinical significance of microfibrils.

Purpose of the Study:

  • To elucidate the molecular basis of fibrillin microfibril assembly.
  • To understand the mechanisms underlying their elastic properties.
  • To clarify the role of microfibrils in higher-order elastic fiber formation.

Main Methods:

  • Analysis of isolated fibrillin-rich microfibrils.
  • Characterization of their 'beads-on-a-string' ultrastructure.

Related Experiment Videos

  • Investigating fibrillin-1's role in microfibril formation and function.

    • Main Results:

    • Fibrillin microfibrils exhibit a distinct 56 nm 'beads-on-a-string' morphology.
    • The molecular assembly and elastic properties are complex and not fully understood.
    • Fibrillin-1 is the primary structural component, and its mutations lead to Marfan syndrome.

    Conclusions:

    • Fibrillin microfibrils are critical for tissue structure and function.
    • Further research is needed to fully understand microfibril assembly and mechanics.
    • Insights into fibrillin microfibrils may lead to therapeutic strategies for Marfan syndrome and related disorders.