Brain lysosomal hydrolases in neuronal ceroid-lipofuscinoses

Molecular and Chemical Neuropathology
V V Prasad, R K Pullarkat

Abstract

Although the neuronal ceroid-lipofuscinoses (NCLs) are often referred to as lysosomal storage disorders, information on brain lysosomal hydrolases in NCLs is not available. We have determined the specific activities of several acid hydrolases in postmortem brain gray matter of infantile (INCL), late infantile (LINCL), juvenile (JNCL), and adult (ANCL) forms of NCL, patients affected with other neurological disorders (ON), and normal controls. The specific activities of beta-hexosaminidase A and B were significantly high in JNCL gray matter, whereas in LINCL, the increase is significant only in beta-hexosaminidase compared to the controls. A significant increase in the activities of alpha-mannosidase, beta-glucuronidase, and acid phosphatase was also observed in LINCL and JNCL patients compared to the control values. beta-galactosidase activity was also found to be elevated in JNCL brains over the controls. In contrast, activities of beta-glucosidase and sialidase appeared to be lowered in INCL and LINCL. On the other hand, alpha-fucosidase, beta-mannosidase, and sulfatase were unaffected in NCLs brains. Thus, the present data indicate NCLs related abnormalities in some of the acid hydrolases in brain gray matter, which are prim...Continue Reading

References

Nov 1, 1977·Journal of Clinical Chemistry and Clinical Biochemistry. Zeitschrift Für Klinische Chemie Und Klinische Biochemie·C M Plum, A Nielsen
Jun 5, 1992·Biochimica Et Biophysica Acta·W Fürst, K Sandhoff
Jan 1, 1988·American Journal of Medical Genetics. Supplement·J A Rider, D L Rider
Jan 1, 1988·American Journal of Medical Genetics. Supplement·N A Hall, A D Patrick
Jan 1, 1988·American Journal of Medical Genetics. Supplement·R K PullarkatV K Patel
Jan 1, 1988·American Journal of Medical Genetics. Supplement·K E WisniewskiJ Heaney-Kieras
Jul 1, 1969·Journal of Neurochemistry·H E Hirsch
Jun 9, 1970·Biochimica Et Biophysica Acta·R O BradyA E Gal
Nov 15, 1984·Clinica Chimica Acta; International Journal of Clinical Chemistry·J BayleranW Saray
Feb 1, 1981·Journal of the Neurological Sciences·P Annunziata, A Federico
Jun 5, 1995·American Journal of Medical Genetics·P Moroni-RawsonT W Jordan
Jun 5, 1995·American Journal of Medical Genetics·K M KhanR K Pullarkat
Apr 24, 1995·Biochimica Et Biophysica Acta·V Gieselmann
Jan 1, 1993·Journal of Inherited Metabolic Disease·R K Pullarkat, S E Zawitosky

❮ Previous
Next ❯

Citations

May 8, 2001·Genetics in Medicine : Official Journal of the American College of Medical Genetics·N ZhongW T Brown
Feb 4, 2006·Neurobiology of Aging·Sabrina S Seehafer, David A Pearce
Sep 26, 2000·Molecular Genetics and Metabolism·N Zhong
Feb 12, 2005·The EMBO Journal·Dagmar KasperThomas J Jentsch
Oct 28, 2005·Human Molecular Genetics·Denia Ramirez-Montealegre, David A Pearce
Nov 30, 2019·Biochimica Et Biophysica Acta. Molecular Basis of Disease·Robert J HuberClaire Russell

❮ Previous
Next ❯

Related Concepts

Related Feeds

Batten Disease

Batten Disease is a group of nervous system disorders known as neuronal ceroid lipofuscinosis. This feed focuses on neurobiological and neuropathological aspects of this disease.