The ataxia related G1107D mutation of the plasma membrane Ca^{2 +} ATPase isoform 3 affects its interplay with calmodulin and the autoinhibition process

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• 作者：Tito Calì ; ^a ; Martina Frizzarin^a ; Laura Luoni^b ; Francesco Zonta^c ; Sergio Pantano^d ; Carlos Cruz^e ; ^d ; Maria Cristina Bonza^b ; Ilenia Bertipaglia^a ; Maria Ruzzene^a ; Maria Ida De Michelis^b ; Nunzio Damiano^a ; Oriano Marin^a ; Ginevra Zanni^f ; Giuseppe Zanotti^a ; Marisa Brini^g ; Raffaele Lopreiato^a ; ^{raffaele.lopreiato@unipd.it} ; Ernesto Carafoli^h ; ^{ernesto.carafoli@unipd.it}
• 关键词：Calcium signaling ; Plasma membrane calcium ATPases ; X-linked cerebellar ataxia ; Calmodulin ; Autoinhibition
• 刊名：Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：1863
• 期：1
• 页码：165-173
• 全文大小：1348 K
• 卷排序：1863

文摘

The ataxia related G1107D mutation of the PMCA 3 impairs its calcium pumping function The mutation affects the interplay of calmodulin with its binding domain on the pump, decreasing its stimulation. The auto inhibitory ability of the calmodulin-binding domain in the resting state is also affected Dissection of the molecular detail of the defect suggests a dual negative effect on the function of the pump