Urinary hyaluronic acid elevation in Hutchinson-Gilford progeria syndrome.

Abstract:

:The basic genetic defect in the Hutchinson-Gilford Progeria Syndrome (progeria), a premature aging syndrome, is unknown. To investigate possible defects in hyaluronic acid (HA) metabolism in this disease, the urinary excretion of HA was studied. Urine specimens from 11 patients with this disorder were examined for HA by a novel high performance liquid chromatography (HPLC) technique. In patients with progeria, HA excretion ranged from 169 micrograms HA/g creatinine to 1440 micrograms HA/g creatinine. In normal age-matched controls, HA excreted ranged from 0 to 77 micrograms HA/g creatinine. In all, a mean 17-fold increase in HA excretion was observed in patients with progeria when compared with age-matched normal controls. Total glycosaminoglycan (GAG) excretion was not elevated. Amongst normal controls, a modest age-related increase in HA excretion was observed. These results suggest that urinary HA levels are abnormally elevated in progeria.

journal_name

Mech Ageing Dev

authors

Zebrower M,Kieras FJ,Brown WT

doi

10.1016/0047-6374(86)90064-3

subject

Has Abstract

pub_date

1986-06-01 00:00:00

pages

39-46

issue

1

eissn

0047-6374

issn

1872-6216

pii

0047-6374(86)90064-3

journal_volume

35

pub_type

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