Sage Journals: Discover world-class research

Abstract

Hydroxyproline metabolism was studied in two patients with type II hyperprolinaemia (HP II) using oral loadings of hydroxyproline or hydroxyproline-ornithine. Δ¹-pyrroline-3-hydroxy-5-carboxylic acid (3 OH-PC) and Δ¹-pyrroline-5-carboxylic acid (PC) were identified in the urine. The urinary excretion of both 3-OH-PC and PC increased in HP II patients but not in healthy controls during oral loading of hydroxyproline and hydroxyproline-ornithine.

The plasma level of proline in patients with HP II is very high but the hydroxyproline concentration is normal or only slightly increased. Therefore one can assume that hydroxyproline is converted to pyrrole-2-carboxylic acid, which is excreted in urine as a glycine conjugate.

In this study it was demonstrated that the highly elevated plasma level of proline in one of the patients with HP II decreased greatly after hydroxyproline-ornithine load; this change was followed by a 40-fold rise in urinary excretion of proline.

References

Adams

(1970). Metabolism of proline and hydroxyproline. International Reviews of Connective Tissue Research, 5, 1–91.

Adams

Goldstone

(1960). Hydroxyproline metabolism. IV. Enzymatic synthesis of δ-hydroxyglutamate from Δ¹-pyrroline-3-hydroxy-5-carboxylate. Journal of Biological Chemistry, 235, 3504–3512.

Applegarth

D. A.

Goodman

S. J.

Irvine

D. G.

Jellum

(1977). Hyperprolinaemia type II: identification of the glycine conjugate of pyrrole-2-carboxylic acid in urine. Clinical Biochemistry, 10, 20–23.

Applegarth

D. A.

Ingram

Hingston

Hardwick

D. F.

(1974). Hyperprolinaemia type II. Clinical Biochemistry, 7, 14–28.

Efron

(1965). Familial hyperprolinaemia. Report of a second case, associated with congenital renal malformations, hereditary hematuria and mild mental retardation, with demonstration of an enzyme defect. New England Journal of Medicine, 272, 1243–1254.

Goodman

S. I.

Mace

J. W.

Miles

B. S.

Teng

C. C.

Brown

S. B.

(1974). Defective hydroxyproline metabolism in type II hyperprolinaemia. Biochemical Medicine, 10, 329–336.

Heacock

A.M.

Adams

(1973). Source of pyrrole-2-carboxylate in mammalian urine. Biochemical and Biophysical Research Communications, 50, 392–397.

Heacock

A. M.

Adams

(1974). Formation and excretion of pyrrole-2-carbocylate in man. Journal of Chemical Investigation, 54, 810–818.

Heacock

A. M.

Adams

(1975). Formation and excretion of pyrrole-2-carboxylic acid. Journal of Biological Chemistry, 250, 2599–2608.

10.

Hotzapple

Genel

Rea

Segal

(1973). Metabolism and uptake of l-proline by human kidney cortex. Pediatric Research, 7, 818–825.

11.

Raine

D. N.

(1975). Treatment by inhibition of renal tubular reabsorption. In The Treatment of Inherited Metabolic Disease, edited by Raine

D. N.

, pp. 219–233. Medical and Technical Publishing, Lancaster.

12.

Scriver

C. R.

Davies

(1965). Endogenous renal clearance rates of free amino acids in pre-pubertal children. Pediatrics, 36, 592–598.

13.

Similä

(1970a). Hyperprolinaemia type II. Annals of Clinical Research, 2, 143–150.

14.

Similä

(1970b). The catabolism of ornithine after intravenous loading in normal subjects and two patients with hyperprolinaemia type II. Clinica Chimica Acta, 28, 457–461.

15.

Valle

Goodman

S. I.

Applegarth

D. A.

Shih

V. E.

Phang

J. M.

(1976). Type II hyperprolinaemia. Δ¹-pyrroline-5-carboxylic acid dehydrogenase deficiency in cultured skin fibroblasts and circulating lymphocytes. Journal of Clinical Investigation, 58, 598–603.

Hydroxyproline Metabolism in Type II Hyperprolinaemia

Abstract

References