The HERC1 E3 Ubiquitin Ligase is essential for normal development and for neurotransmission at the mouse neuromuscular junction

Cellular and Molecular Life Sciences : CMLS
S BachillerR Ruiz

Abstract

The ubiquitin-proteasome system (UPS) plays a fundamental role in protein degradation in neurons, and there is strong evidence that it fulfills a key role in synaptic transmission. The aim of the present work was to study the implication of one component of the UPS, the HERC1 E3 Ubiquitin Ligase, in motor function and neuromuscular transmission. The tambaleante (tbl) mutant mouse carries a spontaneous mutation in HERC1 E3 Ubiquitin Ligase, provoking an ataxic phenotype that develops in the second month of life. Our results show that motor performance in mutant mice is altered at postnatal day 30, before the cerebellar neurodegeneration takes place. This defect is associated with by: (a) a reduction of the motor end-plate area, (b) less efficient neuromuscular activity in vivo, and (c) an impaired evoked neurotransmitter release. Together, these data suggest that the HERC1 E3 Ubiquitin Ligase is fundamental for normal muscle function and that it is essential for neurotransmitter release at the mouse neuromuscular junction.

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Citations

Jan 24, 2016·Cellular and Molecular Life Sciences : CMLS·Susana Sánchez-TenaJose Luis Rosa
Jun 18, 2016·Journal of Neurochemistry·Maria J Pinto, Ramiro D Almeida
Jun 9, 2019·Molecular Neurobiology·Ana DolinarMetka Ravnik-Glavač
Jun 10, 2020·International Journal of Molecular Sciences·Fiona LimanaqiFrancesco Fornai
Sep 10, 2020·International Journal of Molecular Sciences·Sara BachillerRocío Ruiz
Jul 23, 2020·Scientific Reports·Mª Angeles Montes-FernándezJosé A Armengol
Jan 20, 2017·Molecular Neurobiology·Eva Mª Pérez-VillegasJosé A Armengol
Aug 27, 2019·Frontiers in Physiology·Jesús García-CanoJose Luis Rosa
Dec 18, 2020·Frontiers in Neuroanatomy·Eva M Pérez-VillegasJosé A Armengol
Mar 20, 2021·Journal of Intellectual Disability Research : JIDR·E Z TaşkıranG E Utine
Apr 4, 2021·Cancers·Fabiana Alejandra RossiMario Rossi

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