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

Third component of complement in cystic fibrosis.

R J Holzhauer, J D Van Ess, R H Schwartz

    American Journal of Human Genetics
    |November 1, 1976
    PubMed
    Summary

    Elevated complement component 3 (C3) levels were observed in cystic fibrosis (CF) patients and their parents. C3 concentration in CF patients correlated with clinical impairment, suggesting a role in disease severity.

    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

    The cloning of T lymphocytes.

    Immunology today·2014
    Same author

    Comparison of child psychiatry residents' and training directors' perceptions of training for alcohol and substance abuse treatment.

    Academic psychiatry : the journal of the American Association of Directors of Psychiatric Residency Training and the Association for Academic Psychiatry·2014
    Same author

    Treatment of ragweed pollen seasonal allergic conjunctivitis (SAC) with b.i.d. nedocromil sodium 2% ophthalmic solution.

    Ocular immunology and inflammation·2012
    Same author

    TCR engagement in the absence of cell cycle progression leads to T cell anergy independent of p27(Kip1).

    European journal of immunology·2001
    Same author

    N-terminal processing is essential for release of epithin, a mouse type II membrane serine protease.

    The Journal of biological chemistry·2001
    Same author

    Adaptive tolerance of CD4+ T cells in vivo: multiple thresholds in response to a constant level of antigen presentation.

    Journal of immunology (Baltimore, Md. : 1950)·2001

    Area of Science:

    • Immunology
    • Genetics
    • Pediatrics

    Background:

    • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
    • The complement system, including complement component 3 (C3), plays a role in immune responses.
    • Previous research has not fully elucidated the role of C3 in CF pathogenesis.

    Purpose of the Study:

    • To investigate C3 levels and phenotypes in individuals with cystic fibrosis.
    • To determine the correlation between C3 concentration and clinical severity in CF patients.
    • To analyze C3 phenotypes and gene frequencies in CF patients, their families, and healthy controls.

    Main Methods:

    • Quantitative analysis of C3 levels in serum samples.
    • Phenotyping of C3 using established genetic markers.
    • Correlation analysis between C3 levels and the Shwachman-Kulczycki (S-K) score for clinical assessment.
    • Comparison of C3 levels, phenotypes, and gene frequencies across different study groups.

    Main Results:

    • Significant elevations in mean C3 levels were found in CF patients, CF parents, and a specific subgroup of siblings (SS females).
    • C3 concentration in CF patients showed a positive correlation with the degree of clinical impairment (S-K score).
    • No significant differences were observed in C3 phenotype prevalence or S and F gene frequencies among the studied groups.

    Conclusions:

    • Elevated C3 levels are associated with cystic fibrosis and may be inherited from parents.
    • C3 levels correlate with clinical severity in CF patients, suggesting a potential role in disease progression.
    • C3 genetic variations do not appear to be a primary factor in the observed C3 level differences in CF.

    Related Experiment Videos