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Disease Markers
Volume 27, Issue 1, Pages 7-12

Peptide Bound Hypohydroxyprolinuria in Handigodu Disease: A Familial Syndrome of Spondylo Epi(meta)physeal Dysplasia

Mallikarjun Badadani,1 S. V. Suresh Babu,1 K. T. Shetty,1 and S. S. Agarwal2

1Department of Neurochemistry, National Institute of Mental Health and Neuro Sciences, Bangalore-560029, India
2Advanced Center for Treatment Research and Education in Cancer (ACTREC) Navi Mumbai, India

Received 29 September 2009; Accepted 29 September 2009

Copyright © 2009 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Handigodu Disease (HD) is disorder of the osteoarticular system prevalent in few villages of two districts of the state Karnataka in southern India. 24 hrs urinary excretions of proline (Pro) and 4-hydroxyproline (Hyp) were analyzed by HPLC. Decreased peptide bound Hyp excretions (μmole/24 hrs) were found in patient group when compared with controls (Nonaffected; 113.02 ± 67.96, Type-I; 36.22 ± 20.76, Type-II; 45.74 ± 14.95, Type-III; 40.46 ± 22.68) and without significant difference in Pro excretions. Significant increased peptide bound Pro to Hyp ratio were found in patient group compared to control (Nonaffected n = 63: 2.02 ± 1.65, Type-I n = 18: 3.144 ± 1.42, Type-II n = 28: 4.21 ± 1.95, Type-III n = 8: 8.60 ± 6.55). 24 hrs urinary excretions of deoxypyridinoline (DPD) crosslinks were found without significant difference among affected and control, hence HD ruled out from general bone reduction. These results suggest hypohydroxyprolinuria may be because of reduced bone turnover or defective hydroxylation of prolyl residues during post translational modification of collagen biosynthesis.