PMID: 8611653Apr 12, 1996Paper

Evidence for McKusick's hypothesis of deficient collagen cross-linking in patients with homocystinuria

Biochimica Et Biophysica Acta
B LubecG H Boers

Abstract

Osteoporosis occurs commonly in homocystinuria. The underlying pathobiochemical mechanism remains unclear; disturbed cross-linking of collagen has been suggested but this hypothesis has not been fully tested, nor have studies on collagen synthesis been performed. We therefore used recently available noninvasive tests for collagen synthesis and cross-linking to examine 10 patients with homocystinuria. Synthesis of collagen type I and type III was not different from age-matched healthy controls as reflected by comparable plasma levels of carboxyterminal propeptide of type I procollagen (PICP) and of plasma levels of N-terminal propeptide of procollagen type III (PIIINP). Collagen type I cross-links expressed by serum carboxyterminal telopeptide of collagen type I (ICTP) were 1.14 +/- 0.24 micrograms/l in the patient group versus 3.29 +/- 0.32 micrograms/l in the control group. This significant reduction of cross-links in the group with homocystinuria did not correlate with serum homocysteine or homocysteic acid concentrations. Our data clearly indicate that the disturbed cross-linking hypothesis still holds and that the bone manifestations of homocystinuria are not due to deficient collagen synthesis.

References

Feb 1, 1991·Archives of Disease in Childhood·B BartoschG Lubec
Oct 1, 1966·Lancet·E D Harris, A Sjoerdsma
May 18, 1973·Clinica Chimica Acta; International Journal of Clinical Chemistry·S H Jackson
Oct 1, 1973·The Journal of Clinical Investigation·A H Kang, R L Trelstad
Dec 1, 1993·Annals of the Rheumatic Diseases·M HakalaJ Risteli
Feb 1, 1993·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·E F EriksenJ Risteli

❮ Previous
Next ❯

Citations

Aug 10, 2004·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·Morten M VilladsenBente L Langdahl
Sep 1, 2005·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·J GolbaharG R Omrani
Mar 23, 2006·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·M SaitoT Tanaka
Jun 6, 2007·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·M A PérierP D Delmas
Nov 28, 2008·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·K ZhuR L Prince
Jun 2, 2009·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·M SaitoA Shinohara
Sep 18, 2009·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·M Saito, K Marumo
Dec 22, 2011·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·H Wang, C Liu
May 21, 2013·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·J S Lim, D H Lee
Jun 25, 2013·Calcified Tissue International·Tatsuhiko KurodaMasataka Shiraki
Sep 27, 2008·Rheumatology International·Ilhan BayhanGulnur Ozaksit
Oct 11, 2007·Current Osteoporosis Reports·Robert R McLean, Marian T Hannan
Oct 6, 2007·Biological Trace Element Research·Feray SoyupekBurcin Gumus
Feb 24, 2001·Atherosclerosis·M K BodeT Juvonen
Dec 7, 2002·Obstetrics and Gynecology·Sarah E FergusonMark C Walker
Jan 29, 1999·Matrix Biology : Journal of the International Society for Matrix Biology·P CharpiotD Garçon
Dec 13, 2005·The Journals of Gerontology. Series A, Biological Sciences and Medical Sciences·Giovanni RavagliaErminia Mariani
Sep 27, 2005·The Journals of Gerontology. Series A, Biological Sciences and Medical Sciences·Hsu-Ko KuoLewis A Lipsitz
Mar 28, 2008·The Journal of Clinical Endocrinology and Metabolism·Robert R McLeanMarian T Hannan
Apr 1, 1999·Journal of Clinical Densitometry : the Official Journal of the International Society for Clinical Densitometry·Z C Orlic, L G Raisz
Feb 28, 2014·The Proceedings of the Nutrition Society·M ClarkeH McNulty
Jul 23, 2013·Current Osteoporosis Reports·Karin M A SwartPaul Lips
Jun 19, 2014·Molecular and Cellular Biochemistry·Anuradha KalaniNeetu Tyagi
Oct 5, 2011·Nutrition Reviews·Hala Ahmadieh, Asma Arabi
Apr 2, 2015·Nutrients·Valentina Fratoni, Maria Luisa Brandi
May 7, 2013·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·Deniz KiracNesrin Kartal Ozer
Jan 1, 2013·Endocrinología y nutrición : órgano de la Sociedad Española de Endocrinología y Nutrición·Mariangela RondanelliMilena Anna Faliva
Feb 13, 2001·Kidney International. Supplement·A F PernaN G De Santo
Aug 24, 2010·Gastroenterology·Yu-Xiao Yang, David C Metz
Jul 7, 2010·European Journal of Internal Medicine·Paolo BucciarelliPier Mannuccio Mannucci
Apr 14, 2015·Endocrine Journal·Tomohiko Urano, Satoshi Inoue
Feb 17, 2009·Joint, Bone, Spine : Revue Du Rhumatisme·Régis Levasseur
Feb 4, 2009·Drug Discovery Today·Suruchi KhannaDivya Vohora
Dec 11, 2007·Clinical Chemistry and Laboratory Medicine : CCLM·Markus HerrmannWolfgang Herrmann

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.