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Gastrointestinal Dysmotility in MNGIE: from thymidine phosphorylase enzyme deficiency to altered interstitial cells of Cajal

BACKGROUND: MNGIE is a rare and fatal disease in which absence of the enzyme thymidine phosphorylase induces systemic accumulation of thymidine and deoxyuridine and secondary mitochondrial DNA alterations. Gastrointestinal (GI) symptoms are frequently reported in MNGIE patients, however, they are no...

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Dades bibliogràfiques
Publicat a:Orphanet J Rare Dis
Autors principals: Yadak, Rana, Breur, Marjolein, Bugiani, Marianna
Format: Artigo
Idioma:Inglês
Publicat: BioMed Central 2019
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6368792/
https://ncbi.nlm.nih.gov/pubmed/30736844
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s13023-019-1016-6
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