Biometals and glycosylation in humans: Congenital disorders of glycosylation shed lights into the crucial role of Golgi manganese homeostasis

Autor: François Foulquier, Dominique Legrand

Publikováno v: Biochimica et Biophysica Acta (BBA)-General Subjects
Biochimica et Biophysica Acta (BBA)-General Subjects, Elsevier, 2020, 1864 (10), pp.129674. ⟨10.1016/j.bbagen.2020.129674⟩
Biochimica et Biophysica Acta (BBA)-General Subjects, 2020, 1864 (10), pp.129674. ⟨10.1016/j.bbagen.2020.129674⟩

International audience; About half of the eukaryotic proteins bind biometals that participate in their structure and functions in virtually all physiological processes, including glycosylation. After reviewing the biological roles and transport mecha

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::22d5e2431e5420074cf25beb3dada4ba
https://hal.archives-ouvertes.fr/hal-02997375

Altered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosis

Autor: Peter J. Crouch, Grace E. Lidgerwood, Katja M. Kanninen, George Ganio, Irene Volitakis, Aphrodite Caragounis, Alexandra Grubman, Anthony R. White, Clare Duncan, Sarah J. Parker

Publikováno v: Biology Open, Vol 2, Iss 6, Pp 635-646 (2013)
Biology Open

Summary Neuronal ceroid lipofuscinoses, the most common fatal childhood neurodegenerative illnesses, share many features with more prevalent neurodegenerative diseases. Neuronal ceroid lipofuscinoses are caused by mutations in CLN genes. CLN6 encodes

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b8fc51ed280f5202d9bae2b7e340d4b2
http://bio.biologists.org/content/2/6/635

Deregulation of subcellular biometal homeostasis through loss of the metal transporter, Zip7, in a childhood neurodegenerative disorder

Autor: Alexandra Grubman, Jeffrey R. Liddell, Anthony R. White, Laura Bica, Paul S. Donnelly, Shoshanah Longmuir, Malcolm K. Horne, Diane Moujalled, Irene Volitakis, Peter J. Crouch, Grace E. Lidgerwood, Jodi Meyerowitz, James L. Hickey, Imke Tammen, Jari Koistinaho, Aphrodite Caragounis, Clare Duncan, Sarah J. Parker, Jiang-Li Tan, Katja M. Kanninen

Publikováno v: Acta Neuropathologica Communications

Background Aberrant biometal metabolism is a key feature of neurodegenerative disorders including Alzheimer’s and Parkinson’s diseases. Metal modulating compounds are promising therapeutics for neurodegeneration, but their mechanism of action rem

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f321201696110466bd95e9093b954600
https://doi.org/10.1186/2051-5960-2-25

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