Characteristics of the beta-galactosidase-carboxypeptidase complex in GM1-gangliosidosis and beta-galactosialidosis fibroblasts.

Characteristics of the beta-galactosidase-carboxypeptidase complex in GM1-gangliosidosis and beta-galactosialidosis fibroblasts.

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http://www.wikidata.org/entity/Q41610441
Impaired 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.Lysosomal sialidase (neuraminidase-1) is targeted to the cell surface in a multiprotein complex that facilitates elastic fiber assemblyA comparative study of cytoplasmic granules imaged by the real-time microscope, Nile Red and Filipin in fibroblasts from patients with lipid storage diseases.Protective protein/cathepsin A down-regulates osteoclastogenesis by associating with and degrading NF-kappaB p50/p65.Mutations in sialidosis impair sialidase binding to the lysosomal multienzyme complex.Identification of Glu-268 as the catalytic nucleophile of human lysosomal beta-galactosidase precursor by mass spectrometry.Cathepsin A deficiency in galactosialidosis: studies of patients and carriers in 16 families.The 67-kD elastin/laminin-binding protein is related to an enzymatically inactive, alternatively spliced form of beta-galactosidase.Human placental beta-galactosidase. Characterization of the dimer and complex forms of the enzyme.Kinetic mechanism and characterization of human beta-galactosidase precursor secreted by permanently transfected Chinese hamster ovary cellsWhole mount enzyme histochemistry as a rapid screen at necropsy for expression of beta-galactosidase (LacZ)-bearing transgenes: considerations for separating specific LacZ activity from nonspecific (endogenous) galactosidase activity.Review
P2860

Characteristics of the beta-galactosidase-carboxypeptidase complex in GM1-gangliosidosis and beta-galactosialidosis fibroblasts.

Item
http://www.wikidata.org/entity/Q41610441
description
1992 nî lūn-bûn
@nan
1992年の論文
@ja
1992年論文
@yue
1992年論文
@zh-hant
1992年論文
@zh-hk
1992年論文
@zh-mo
1992年論文
@zh-tw
1992年论文
@wuu
1992年论文
@zh
1992年论文
@zh-cn
name
Characteristics of the beta-ga ...... galactosialidosis fibroblasts.
@en
type
label
Characteristics of the beta-ga ...... galactosialidosis fibroblasts.
@en
prefLabel
Characteristics of the beta-ga ...... galactosialidosis fibroblasts.
@en
P1433
P1476
P2093
P2860
P304
P31
P356
P407
P478
P577
P5875
P698
P932
P356
P1433
P1476
Characteristics of the beta-ga ...... galactosialidosis fibroblasts.
@en
P2093
P2860
P304
P31
P356
P407
P478
P577
P5875
P698
P932