Skip to main content
main-content

01.11.2007 | Original Article | Ausgabe 6/2007 Open Access

Journal of Inherited Metabolic Disease 6/2007

Advanced glycation end products and the absence of premature atherosclerosis in glycogen storage disease Ia

Zeitschrift:
Journal of Inherited Metabolic Disease > Ausgabe 6/2007
Autoren:
N. C. den Hollander, D. J. Mulder, R. Graaff, S. R. Thorpe, J. W. Baynes, G. P. A. Smit, A. J. Smit
Wichtige Hinweise
Communicating editor: René Santer
Competing interests: R. Graaff and A.J. Smit are founders of DiagnOptics B.V., The Netherlands, manufacturer of the AGE- Reader
References to electronic databases: Glycogen storage disease type Ia (GSD Ia): OMIM +232200. Glucose-6-phosphatase (G6Pase) EC 3.1.3.9.

Summary

Introduction

Despite their unfavourable cardiovascular risk profile, patients with glycogen storage disease type Ia (GSD Ia) do not develop premature atherosclerosis. We hypothesized that this paradox might be related to a decreased formation of advanced glycation end products (AGEs) resulting from lifetime low plasma glucose levels and decreased oxidative stress.

Methods

In 8 GSD Ia patients (age 20–24 years) and 30 matched controls we measured carotid intima-media thickness (IMT), skin autofluorescence (AF; a non-invasive index for AGEs), and specific AGEs (pentosidine, N-(carboxymethyl)lysine (CML), N-(carboxyethyl)lysine (CEL)) and collagen linked fluorescence (CLF, measured at excitation/emission wavelength combinations of 328/378 and 370/440 nm) in skin samples.

Results

Carotid IMT was significantly lower in GSD Ia patients. Skin AF did not differ between patients and controls. The skin samples showed higher CEL levels in the patient group (p=0.008), but similar levels of pentosidine, CML, and CLF. In the total group, skin AF correlated with CML (r=0.39, p=0.031), CLF 328/378 nm (r=0.53; p=0.002) and CLF 370/440 nm (r=0.60; p=0.001). In the control group, AF also correlated with the maximum carotid IMT (r=0.6; p=0.004).

Conclusion

Although our data confirm that GSD Ia patients present with a reduced burden of atherosclerosis, this phenomenon cannot be explained by differences in AGE accumulation as measured in the skin.

Unsere Produktempfehlungen

e.Med Interdisziplinär

Kombi-Abonnement

Für Ihren Erfolg in Klinik und Praxis - Die beste Hilfe in Ihrem Arbeitsalltag als Mediziner

Mit e.Med Interdisziplinär erhalten Sie Zugang zu allen CME-Fortbildungen und Fachzeitschriften auf SpringerMedizin.de.

Alle e.Med Abos bis 30. April 2021 zum halben Preis!

Jetzt e.Med zum Sonderpreis bestellen!

Literatur
Über diesen Artikel

Weitere Artikel der Ausgabe 6/2007

Journal of Inherited Metabolic Disease 6/2007 Zur Ausgabe

Neu im Fachgebiet Innere Medizin

Mail Icon II Newsletter

Bestellen Sie unseren kostenlosen Newsletter Update Innere Medizin und bleiben Sie gut informiert – ganz bequem per eMail.

© Springer Medizin 

Bildnachweise