Biochem Int. 1991 May;24(2):209-15.

Human N-acetylgalactosamine-4-sulphatase: protein maturation and isolation of genomic clones.

Litjens T, Morris CP, Gibson GJ, Beckmann KR, Hopwood JJ.

Source

Department of Chemical Pathology, Adelaide Children's Hospital, S.A., Australia.

Abstract

N-Acetylgalactosamine-4-sulphatase (EC 3.1.6.1, G4S) is composed of a 57 kDa species in human liver that dissociates into 43 kDa and 8 kDa subunits under reducing conditions and, when deficient, causes the lysosomal storage disorder, mucopolysaccharidosis type VI. We isolated genomic clones containing the G4S first exon, including the leader peptide and the amino terminus of the 43 kDa polypeptide. Amino-terminal amino acid sequences of the 43 kDa and 8 kDa subunits indicated that the 8 kDa component is linked to the 43 kDa polypeptide by a single disulphide bond, does not contain the mannose-6-phosphate lysosomal targeting signal and is at the carboxyl terminus of G4S.

PMID:: 1930244; [PubMed - indexed for MEDLINE]

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Identification, expression, and biochemical characterization of N-acetylgalactosamine-4-sulfatase mutations and relationship with clinical phenotype in MPS-VI patients. [Am J Hum Genet. 1996]
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Characterization of a deletion at Xq27-q28 associated with unbalanced inactivation of the nonmutant X chromosome. [Am J Hum Genet. 1992]
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Human N-acetylgalactosamine-4-sulphatase: protein maturation and isolation of genomic clones.

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