A Mouse Model Suggests Two Mechanisms for Thyroid Alterations in Infantile Cystinosis: Decreased Thyroglobulin Synthesis Due to Endoplasmic Reticulum Stress/Unfolded Protein Response and Impaired Lysosomal Processing

Thyroid hormones are released from thyroglobulin (Tg) in lysosomes, which are impaired in infantile/nephropathic cystinosis. Cystinosis is a lysosomal cystine storage disease due to defective cystine exporter, cystinosin. Cystinotic children develop subclinical and then overt hypothyroidism. Why hyp...

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Pubblicato in:Endocrinology
Autori principali: Gaide Chevronnay, H. P., Janssens, V., Van Der Smissen, P., Liao, X. H., Abid, Y., Nevo, N., Antignac, C., Refetoff, S., Cherqui, S., Pierreux, C. E., Courtoy, P. J.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Endocrine Society 2015
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Original Research
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4430621/
https://ncbi.nlm.nih.gov/pubmed/25811319
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1210/en.2014-1672
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