Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

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A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease.Alpha-Mannosidosis: Therapeutic Strategies.

P2860

Q41285403-243A35C3-38D3-4574-B7A1-2A9E65ED8DD9 Q55007551-5B276171-A8E4-401E-B77B-F0C53B4BAEA7

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Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

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http://www.wikidata.org/entity/Q36410390

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2015 nî lūn-bûn

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2015年の論文

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2015年学术文章

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Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

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Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

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type

Item

label

Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

@ast

Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

@en

prefLabel

Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

@ast

Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

@en

P1476

Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

@en

P2093

Annika Ericsson

http://www.w3.org/2001/XMLSchema#string

Claes Andersson

http://www.w3.org/2001/XMLSchema#string

Hans Christian Beck

http://www.w3.org/2001/XMLSchema#string

Jens Fogh

http://www.w3.org/2001/XMLSchema#string

Judith Blanz

http://www.w3.org/2001/XMLSchema#string

Markus Damme

http://www.w3.org/2001/XMLSchema#string

Meike Lüdemann

http://www.w3.org/2001/XMLSchema#string

Michelle Rothaug

http://www.w3.org/2001/XMLSchema#string

Paul Saftig

http://www.w3.org/2001/XMLSchema#string

Renate Lüllmann-Rauch

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P2860

Alpha-mannosidosis

Missense and nonsense mutations in the lysosomal alpha-mannosidase gene (MANB) in severe and mild forms of alpha-mannosidosis

Human and mouse microglia express connexin36, and functional gap junctions are formed between rodent microglia and neurons

A phase 1/2 clinical trial of enzyme replacement in fabry disease: pharmacokinetic, substrate clearance, and safety studies

Enzyme replacement therapy in a mouse model of aspartylglycosaminuria

Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis

Dose translation from animal to human studies revisited

Cerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in α-mannosidosis.

Impaired lysosomal trimming of N-linked oligosaccharides leads to hyperglycosylation of native lysosomal proteins in mice with alpha-mannosidosis.

Glycoprotein lysosomal storage disorders: alpha- and beta-mannosidosis, fucosidosis and alpha-N-acetylgalactosaminidase deficiency.

P304

987-1001

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P31

scholarly article

P356

10.1002/ACN3.245

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P433

http://www.w3.org/2001/XMLSchema#string

P478

http://www.w3.org/2001/XMLSchema#string

P50

Renate Lüllmann-Rauch

P577

2015-09-19T00:00:00Z

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P5875

281963356

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P698

26817023

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P921

Enzyme replacement therapy

P932

4693626

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Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

about

P2860

P2860

Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice

description

name

type

label

prefLabel

P1433

P1476

P2093

P2860

P304

P31

P356

P433

P478

P50

P577

P5875

P698

P921

P932

P356

P1433

P1476

P2093

P2860

P304

P31

P356

P433

P478

P50

P577

P5875

P698

P921

P932