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Titolo:
THE PRO-PEPTIDE OF THE PRO-BETA-POLYPEPTIDE CHAIN OF HUMAN BETA-HEXOSAMINIDASE IS NECESSARY FOR PROPER PROTEIN-FOLDING AND EXIT FROM THE ENDOPLASMIC-RETICULUM
Autore:
SAGHERIAN C; THORNER P; MAHURAN D;
Indirizzi:
HOSP SICK CHILDREN,RES INST,555 UNIV AVE TORONTO M5G 1X8 ON CANADA HOSP SICK CHILDREN,RES INST TORONTO M5G 1X8 ON CANADA UNIV TORONTO,DEPT CLIN BIOCHEM TORONTO ON CANADA UNIV TORONTO,DEPT PATHOL TORONTO ON CANADA
Titolo Testata:
Biochemical and biophysical research communications
fascicolo: 1, volume: 204, anno: 1994,
pagine: 135 - 141
SICI:
0006-291X(1994)204:1<135:TPOTPC>2.0.ZU;2-Z
Fonte:
ISI
Lingua:
ENG
Soggetto:
TAY-SACHS-DISEASE; ALPHA-SUBUNIT; HEXB GENE; N-ACETYLHEXOSAMINIDASE; EXTENSIVE HOMOLOGY; HUMAN-FIBROBLASTS; SEQUENCE-ANALYSIS; LYSOSOMAL-ENZYME; CDNA; MUTATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
36
Recensione:
Indirizzi per estratti:
Citazione:
C. Sagherian et al., "THE PRO-PEPTIDE OF THE PRO-BETA-POLYPEPTIDE CHAIN OF HUMAN BETA-HEXOSAMINIDASE IS NECESSARY FOR PROPER PROTEIN-FOLDING AND EXIT FROM THE ENDOPLASMIC-RETICULUM", Biochemical and biophysical research communications, 204(1), 1994, pp. 135-141

Abstract

We examine the function of the pro beta-peptide (residues 42-121) in the folding and intracellular transport of human beta-hexosaminidase B(beta-N-acetylhexosaminidase, EC 3.2.1.52). A construct was prepared that encoded an in frame deletion of residues 55-118. Expression of this construct in COS-1 cells produced a beta-polypeptide chain that formed insoluble aggregates and remained trapped in the endoplasmic reticulum (ER). We conclude that the pro beta-peptide may act as a type of intramolecular chaperone for the mature beta-subunit. (C) 1994 Academic Press, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/11/20 alle ore 13:50:17