2022
XBP1 Activation Reduces Severity of Polycystic Kidney Disease due to a Nontruncating Polycystin-1 Mutation in Mice
Krappitz M, Bhardwaj R, Dong K, Staudner T, Yilmaz DE, Pioppini C, Westergerling P, Ruemmele D, Hollmann T, Nguyen TA, Cai Y, Gallagher AR, Somlo S, Fedeles S. XBP1 Activation Reduces Severity of Polycystic Kidney Disease due to a Nontruncating Polycystin-1 Mutation in Mice. Journal Of The American Society Of Nephrology 2022, 34: 110-121. PMID: 36270750, PMCID: PMC10101557, DOI: 10.1681/asn.2021091180.Peer-Reviewed Original ResearchConceptsPolycystin-1Polycystin-2Functional polycystin-1Amino acid substitution mutationsAutosomal dominant polycystic kidney diseaseIntegral membrane proteinsTranscription factor XBP1Unfolded protein responsePost-translational maturationAcid substitution mutationsEndoplasmic reticulum chaperoneCiliary traffickingXBP1 activityChaperone functionIntegral membraneActive XBP1Polycystic kidney diseaseMembrane proteinsPC1 functionsPrimary ciliaProtein responseHypomorphic mutationsTransgenic activationSubstitution mutationsTransgenic expression
1988
Biosynthesis of thyrotropin-releasing hormone by a rat medullary thyroid carcinoma cell line.
Sevarino K, Wu P, Jackson I, Roos B, Mandel G, Goodman R. Biosynthesis of thyrotropin-releasing hormone by a rat medullary thyroid carcinoma cell line. Journal Of Biological Chemistry 1988, 263: 620-623. PMID: 3121619, DOI: 10.1016/s0021-9258(19)35397-9.Peer-Reviewed Original ResearchConceptsHypothalamic tissueCA77 cellsThyrotropin-releasing hormoneThyroid carcinoma cell linesRat medullary thyroid carcinoma cell lineMedullary thyroid carcinoma cell lineHormone messenger RNAPost-translational maturationCarcinoma cell linesImmunoreactive TRHRat hypothalamusPromising model systemGene regulationTRH precursorFurther studiesCell linesMessenger RNACell processesModel systemRNATissueRelative levels
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