Galactosialidosis: simultaneous deficiency of esterase, carboxy-terminal deamidase and acid carboxypeptidase activities (Q24309596)

carboxypeptidase activity

GOA release 2020-03-11

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GOA release 2020-03-11

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R Kase

1

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K Itoh

2

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N Takiyama

3

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A Oshima

4

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H Sakuraba

5

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Y Suzuki

Biochemical and Biophysical Research Communications

6

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language of work or name

English

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publication date

15 November 1990

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published in

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volume

172

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issue

3

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page(s)

1175-9

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cites work

beta-Galactosidase deficiency in juvenile and adult patients. Report of six Japanese cases and review of literature

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A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding

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Mucolipidosis I: increased sialic acid content and deficiency of an alpha-N-acetylneuraminidase in cultured fibroblasts

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Macular cherry-red spots and myoclonus with dementia: Coexistent neuraminidase and β-galactosidase deficiencies

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Deficient lysosomal carboxypeptidase activity in galactosialidosis

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Galactosialidosis (beta-galactosidase-neuraminidase deficiency): a possible role of serine-thiol proteases in the degradation of beta-galactosidase molecules

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Galactosialidosis: a direct evidence that a 46-kilodalton protein restores deficient enzyme activities in fibroblasts

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7 January 2021

Expression of cDNA encoding the human "protective protein" associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases

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Gene dosage-dependent secretion of yeast vacuolar carboxypeptidase Y.

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Human placental neuraminidase. Activation, stabilization and association with beta-galactosidase and its protective protein

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Generalized gangliosidosis: beta-galactosidase deficiency.

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