Naslov
Kinetics and activity of arylsulfatase A in leukocytes derived from patients with cerebral palsy

Autori
Kalanj Bognar, Svjetlana ; Foretić, Blaženka ; Furač, Ivana ; Vukelić, Željka ; Grubešić, Zdravko

Izvornik
Acta Pharmaceutica (1330-0075) 56 (2006), 1; 95-104

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
arylsulfatase A; kinetic parameters; enzyme activity; mutations in ASA gene; cerebral palsy

Sažetak
Activity and kinetics of arylsulfatase A (ASA, EC 3.1.6.8) were analyzed in leukocyte homogenates derived from patients suffering from cerebral palsy. Lower ASA activity was found in the patients leukocytes, as determined by spectrophotometry using chromogenic substrate p-nitrocatechol sulfate (p-NCS). Kinetic parameters, Km and vmax, for leukocyte ASA were determined from the dependence of initial reaction velocities on the p-NCS concentrations. A slight difference in Km values was found for leukocyte enzyme in cerebral palsy (0.26 mmol L-1) as compared with control (0.21 mmol L-1), whereas vmax value for the leukocyte ASA in disease reached only 58% of the control value. In addition, the presence of the most common mutations associated with ASA pseudodeficiency (N350S, 1524+95 A>G) and metachromatic leukodystrophy (P426L) was detected in all investigated patients. Changes in activity and kinetic parameters of leukocyte ASA in cerebral palsy are most probably related to the decrease of enzyme concentration ; the detected mutations might at least partially contribute to the observed changes.

Izvorni jezik
Engleski

Znanstvena područja
Kemija, Temeljne medicinske znanosti

POVEZANOST RADA