Christopher G Wier1, Alexander E Crum2, Anthony B Reynolds1, Chitra C Iyer2, Deepti Chugh2, Marilly S Palettas3, Patrick L Heilman2, David M Kline3, W David Arnold2,4,5,6, Stephen J Kolb1,2. 1. Department of Biological Chemistry and Pharmacology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA. 2. Department of Neurology, Division of Neuromuscular Medicine, The Ohio State University Wexner Medical Center, 395 West 12th Avenue, Columbus, Ohio, 43210, USA. 3. Center for Biostatistics, Department of Biomedical Informatics, The Ohio State University, Columbus, Ohio, USA. 4. Department of Physical Medicine and Rehabilitation, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA. 5. Department of Neuroscience, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA. 6. Department of Physiology and Cell Biology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.
Abstract
INTRODUCTION: Electrophysiological measurements are used in longitudinal clinical studies to provide insight into the progression of amyotrophic lateral sclerosis (ALS) and the relationship between muscle weakness and motor unit (MU) degeneration. Here, we used a similar longitudinal approach in the Cu/Zn superoxide dismutase (SOD1[G93A]) mouse model of ALS. METHODS: In vivo muscle contractility and MU connectivity assays were assessed longitudinally in SOD1(G93A) and wild type mice from postnatal days 35 to 119. RESULTS: In SOD1(G93A) males, muscle contractility was reduced by day 35 and preceded MU loss. Muscle contractility and motor unit reduction were delayed in SOD1(G93A) females compared with males, but, just as with males, muscle contractility reduction preceded MU loss. DISCUSSION: The longitudinal contractility and connectivity paradigm employed here provides additional insight into the SOD1(G93A) mouse model and suggests that loss of muscle contractility is an early finding that may precede loss of MUs and motor neuron death. Muscle Nerve 59:254-262, 2019.
INTRODUCTION: Electrophysiological measurements are used in longitudinal clinical studies to provide insight into the progression of amyotrophic lateral sclerosis (ALS) and the relationship between muscle weakness and motor unit (MU) degeneration. Here, we used a similar longitudinal approach in the Cu/Zn superoxide dismutase (SOD1[G93A]) mouse model of ALS. METHODS: In vivo muscle contractility and MU connectivity assays were assessed longitudinally in SOD1(G93A) and wild type mice from postnatal days 35 to 119. RESULTS: In SOD1(G93A) males, muscle contractility was reduced by day 35 and preceded MU loss. Muscle contractility and motor unit reduction were delayed in SOD1(G93A) females compared with males, but, just as with males, muscle contractility reduction preceded MU loss. DISCUSSION: The longitudinal contractility and connectivity paradigm employed here provides additional insight into the SOD1(G93A) mouse model and suggests that loss of muscle contractility is an early finding that may precede loss of MUs and motor neuron death. Muscle Nerve 59:254-262, 2019.
Authors: Clifton L Gooch; Timothy J Doherty; K Ming Chan; Mark B Bromberg; Richard A Lewis; Dan W Stashuk; Michael J Berger; Michael T Andary; Jasper R Daube Journal: Muscle Nerve Date: 2014-12 Impact factor: 3.217
Authors: Anson Cheng; Marco Morsch; Yui Murata; Nazanin Ghazanfari; Stephen W Reddel; William D Phillips Journal: PLoS One Date: 2013-07-03 Impact factor: 3.240
Authors: Carlos J Padilla; Markus E Harrigan; Hallie Harris; Jan M Schwab; Seward B Rutkove; Mark M Rich; Brian C Clark; W David Arnold Journal: Geroscience Date: 2021-04-24 Impact factor: 7.713
Authors: Deepti Chugh; Chitra C Iyer; Xueyong Wang; Prameela Bobbili; Mark M Rich; W David Arnold Journal: Neurobiol Aging Date: 2019-11-05 Impact factor: 4.673
Authors: Deepti Chugh; Chitra C Iyer; Prameela Bobbili; Anton J Blatnik; Brian K Kaspar; Kathrin Meyer; Arthur Hm Burghes; Brian C Clark; W David Arnold Journal: Neurobiol Aging Date: 2021-02-05 Impact factor: 4.673
Authors: Chitra C Iyer; Deepti Chugh; Prameela J Bobbili; Anton J Blatnik Iii; Alexander E Crum; Allen F Yi; Brian K Kaspar; Kathrin C Meyer; Arthur H M Burghes; W David Arnold Journal: Neurobiol Aging Date: 2021-03-12 Impact factor: 5.133
Authors: Markus E Harrigan; Angela R Filous; Andrew P Tosolini; Renee Morris; Jan M Schwab; W David Arnold Journal: Sci Rep Date: 2019-11-13 Impact factor: 4.379
Authors: Maria H H Balch; Hallie Harris; Deepti Chugh; Surya Gnyawali; Cameron Rink; Shahid M Nimjee; W David Arnold Journal: Exp Neurol Date: 2021-05-25 Impact factor: 5.620