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

Glomerular basement membrane changes in hereditary glomerular diseases

M C Gubler, M Levy, C Naizot

    Renal Physiology
    |January 1, 1980
    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

    Very early twin-to-twin transfusion syndrome and discordant activation of the renin-angiotensin system.

    Placenta·2009
    Same author

    Autosomal dominant Alport's syndrome: study of a large Tunisian family.

    Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia·2006
    Same author

    Meckel-Grüber syndrome: sonography and pathology.

    Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology·2006
    Same author

    Sonographic findings in Beckwith-Wiedemann syndrome related to H19 hypermethylation.

    Prenatal diagnosis·2004
    Same author

    Prenatal diagnosis of bilateral isolated fetal hyperechogenic kidneys. Is it possible to predict long term outcome?

    BJOG : an international journal of obstetrics and gynaecology·2002
    Same author

    [Contribution of genetics to knowledge and management of hereditary kidney diseases progressing to renal failure].

    Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2001
    Same journal

    Effect of substrate-free albumin on perfused rat kidney function.

    Renal physiology·1988
    Same journal

    Effect of maleate on tubular protein reabsorption in dog kidneys.

    Renal physiology·1988
    Same journal

    Outflow segment of the efferent arteriole of the rat glomerulus investigated by in vivo and electron microscopy.

    Renal physiology·1988
    Same journal

    Aminoglycoside and nephrotoxicity.

    Renal physiology·1988
    Same journal

    Intrarenal kallikrein-kinin activity in acute renovascular hypertension in dogs.

    Renal physiology·1988
    Same journal

    Complex physiological and biochemical action of aldosterone in toad urinary bladder and mammalian renal collecting duct cells.

    Renal physiology·1988
    See all related articles

    Ultrastructural changes in glomerular basement membranes are key diagnostic markers for hereditary kidney diseases like Alport syndrome. Understanding these changes helps identify biochemical defects and guides further research.

    Area of Science:

    • Nephrology
    • Pathology
    • Genetics

    Background:

    • Hereditary glomerular diseases often manifest with ultrastructural abnormalities of the glomerular basement membrane (GBM).
    • Specific GBM changes, such as thickening, thinning, or fibril presence, are associated with distinct genetic kidney disorders.

    Purpose of the Study:

    • To highlight the diagnostic utility of ultrastructural GBM changes in hereditary glomerular diseases.
    • To underscore the link between GBM morphology and underlying biochemical defects.
    • To emphasize the need for further research into the molecular basis of these conditions.

    Main Methods:

    • Ultrastructural examination of kidney biopsies.
    • Correlation of morphological findings with specific hereditary glomerular diseases (Alport syndrome, familial benign essential hematuria, nail-patella syndrome).

    Related Experiment Videos

    Main Results:

    • Alport syndrome shows thick/thin GBM with lamina densa splitting.
    • Familial benign essential hematuria is characterized by a thin GBM.
    • Nail-patella syndrome presents with a thick GBM and collagen-like fibrils.

    Conclusions:

    • Ultrastructural GBM changes serve as valuable diagnostic markers for hereditary glomerular diseases.
    • These morphological findings point towards primary defects in GBM metabolism.
    • Further biochemical and immunochemical studies are essential for a comprehensive understanding.