Human β-glucuronidase: structure, function, and application in enzyme replacement therapy.

Abstract:

:Lysosomal storage diseases occur due to incomplete metabolic degradation of macromolecules by various hydrolytic enzymes in the lysosome. Despite structural differences, most of the lysosomal enzymes share many common features including a lysosomal targeting motif and phosphotransferase recognition sites. β-Glucuronidase (GUSB) is an important lysosomal enzyme involved in the degradation of glucuronate-containing glycosaminoglycan. The deficiency of GUSB causes mucopolysaccharidosis type VII (MPSVII), leading to lysosomal storage in the brain. GUSB is a well-studied protein for its expression, sequence, structure, and function. The purpose of this review is to summarize our current understanding of sequence, structure, function, and evolution of GUSB and its lysosomal enzyme targeting. Enzyme replacement therapy reported for this protein is also discussed.

journal_name

Rejuvenation Res

journal_title

Rejuvenation research

authors

Naz H,Islam A,Waheed A,Sly WS,Ahmad F,Hassan I

doi

10.1089/rej.2013.1407

subject

Has Abstract

pub_date

2013-10-01 00:00:00

pages

352-63

issue

5

eissn

1549-1684

issn

1557-8577

journal_volume

16

pub_type

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