Cerebellar Alterations and Gait Defects as Therapeutic Outcome Measures for Enzyme Replacement Therapy in α-Mannosidosis

Author

DAMME, Markus¹ ; STROOBANTS, Stijn⁴ ; WALKLEY, Steven U⁵ ; LÜLLMANN-RAUCH, Renate⁶ ; D'HOOGE, Rudi⁴ ; FOGH, Jens⁷ ; SAFTIG, Paul³ ; LÜBKE, Torben² ; BLANZ, Judith³
[1] Department of Biochemistry 2 Georg-August University, Göttingen, Germany
[2] Department of Biochemistry 1 University of Bielefeld, Germany
[3] Biochemical Institute University of Kiel, Germany
[4] Laboratory of Biological Psychology University of Leuven, Belgium
[5] Sidney Weisner Laboratory of Genetic Neurological Disease, Department of Neuroscience Rose F. Kennedy Center for Research in Mental Retardation and Human Development, Albert Einstein College of Medicine, Bronx, New York, United States
[6] Anatomisches Institut , Christian-Albrechts University Kiel, Germany
[7] Zymenex A/S, Hillerod, Denmark

Source

Journal of neuropathology and experimental neurology. 2011, Vol 70, Num 1, pp 83-94, 12 p ; ref : 54 ref

CODEN

JNENAD

ISSN

0022-3069

Scientific domain

Neurology

Publisher

Lippincott Williams & Wilkins, Hagerstown, MD

Publication country

United States

Document type

Article

Language

English

Author keyword

Cerebellar atrophy Enzyme replacement therapy Gait defects Knockout mouse model Lysosomal storage disease α-Mannosidase α-Mannosidosis

Keyword (fr)

Allure Altération Atrophie Cervelet Enzyme Glucide Mannosidose Modèle animal Mutation Pathologie des lysosomes Pathologie du système nerveux Pronostic Souris Traitement substitutif Encéphale Enzymopathie Maladie héréditaire Maladie métabolique Mammalia Mucolipidose Rodentia Système nerveux central Vertebrata

Keyword (en)

Gait Alteration Atrophy Cerebellum Enzyme Carbohydrate Mannosidosis Animal model Mutation Lysosomal storage disease Nervous system diseases Prognosis Mouse Replacement therapy Encephalon Enzymopathy Genetic disease Metabolic diseases Mammalia Mucolipidosis Rodentia Central nervous system Vertebrata

Keyword (es)

Marcha Alteración Atrofia Cerebelo Enzima Glúcido Manosidosis Modelo animal Mutación Lisosoma patología Sistema nervioso patología Pronóstico Ratón Tratamiento sustitutivo Encéfalo Enzimopatía Enfermedad hereditaria Metabolismo patología Mammalia Mucolipidosis Rodentia Sistema nervioso central Vertebrata

Classification

Pascal

002 Biological and medical sciences / 002B Medical sciences / 002B17 Neurology

Discipline

Neurology

Origin

Inist-CNRS

Database

PASCAL

INIST identifier

23740801

Sauf mention contraire ci-dessus, le contenu de cette notice bibliographique peut être utilisé dans le cadre d’une licence CC BY 4.0 Inist-CNRS / Unless otherwise stated above, the content of this bibliographic record may be used under a CC BY 4.0 licence by Inist-CNRS / A menos que se haya señalado antes, el contenido de este registro bibliográfico puede ser utilizado al amparo de una licencia CC BY 4.0 Inist-CNRS