Early proteolytic cleavage with loss of a C-terminal fragment underlies altered processing of the beta-galactosidase precursor in galactosialidosis
about
Molecular mechanism of lysosomal sialidase deficiency in galactosialidosis involves its rapid degradationImpaired elastic-fiber assembly by fibroblasts from patients with either Morquio B disease or infantile GM1-gangliosidosis is linked to deficiency in the 67-kD spliced variant of beta-galactosidase.Role of beta-galactosidase and elastin binding protein in lysosomal and nonlysosomal complexes of patients with GM1-gangliosidosisThe 67-kDa enzymatically inactive alternatively spliced variant of beta-galactosidase is identical to the elastin/laminin-binding proteinAssociation of N-acetylgalactosamine-6-sulfate sulfatase with the multienzyme lysosomal complex of beta-galactosidase, cathepsin A, and neuraminidase. Possible implication for intralysosomal catabolism of keratan sulfateMutations in sialidosis impair sialidase binding to the lysosomal multienzyme complex.Molecular consequences of the pathogenic mutation in feline GM1 gangliosidosisIdentification of Glu-268 as the catalytic nucleophile of human lysosomal beta-galactosidase precursor by mass spectrometry.Primary and secondary elastin-binding protein defect leads to impaired elastogenesis in fibroblasts from GM1-gangliosidosis patients.Fluorous iminoalditols act as effective pharmacological chaperones against gene products from GLB₁ alleles causing GM1-gangliosidosis and Morquio B disease.Maturation and degradation of beta-galactosidase in the post-Golgi compartment are regulated by cathepsin B and a non-cysteine protease.Insights into post-translational processing of beta-galactosidase in an animal model resembling late infantile human G-gangliosidosis.Characterization of beta-galactosidase mutations Asp332-->Asn and Arg148-->Ser, and a polymorphism, Ser532-->Gly, in a case of GM1 gangliosidosisGeneration and characterization of recombinant feline beta-galactosidase for preclinical enzyme replacement therapy studies in GM1 gangliosidosis.
P2860
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P2860
Early proteolytic cleavage with loss of a C-terminal fragment underlies altered processing of the beta-galactosidase precursor in galactosialidosis
description
1996 nî lūn-bûn
@nan
1996年の論文
@ja
1996年論文
@yue
1996年論文
@zh-hant
1996年論文
@zh-hk
1996年論文
@zh-mo
1996年論文
@zh-tw
1996年论文
@wuu
1996年论文
@zh
1996年论文
@zh-cn
name
Early proteolytic cleavage wit ...... precursor in galactosialidosis
@en
Early proteolytic cleavage wit ...... precursor in galactosialidosis
@nl
type
label
Early proteolytic cleavage wit ...... precursor in galactosialidosis
@en
Early proteolytic cleavage wit ...... precursor in galactosialidosis
@nl
prefLabel
Early proteolytic cleavage wit ...... precursor in galactosialidosis
@en
Early proteolytic cleavage wit ...... precursor in galactosialidosis
@nl
P2093
P2860
P356
P1433
P1476
Early proteolytic cleavage wit ...... precursor in galactosialidosis
@en
P2093
P2860
P304
P356
10.1042/BJ3130787
P407
P478
313 ( Pt 3)
P577
1996-02-01T00:00:00Z