ORCID Profile
0000-0002-7203-4520
Current Organisations
Shirley Ryan AbilityLab
,
Northwestern University - Chicago
,
Edward Hines Junior VA Hospital
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Publisher: Wiley
Date: 11-12-2013
DOI: 10.1002/MUS.23927
Abstract: We investigated the contribution of muscle fibrosis to elbow flexion contractures in a murine model of neonatal brachial plexus injury (NBPI). Four weeks after NBPI, biceps and brachialis fibrosis were assessed histologically and compared with the timing of contracture development and the relative contribution of each muscle to contractures. Modulus of elasticity and hydroxyproline (collagen) content were measured and correlated with contracture severity. The effect of halofuginone antifibrotic therapy on fibrosis and contractures was investigated. Elbow contractures preceded muscle fibrosis development. The brachialis was less fibrotic than the biceps, yet contributed more to contractures. Modulus and hydroxyproline content increased in both elbow flexors, but neither correlated with contracture severity. Halofuginone reduced biceps fibrosis but did not reduce contracture severity. Contractures after NBPI cannot be explained solely by muscle fibrosis, arguing for investigation of alternate pathophysiologic targets for contracture prevention and treatment.
Publisher: Elsevier BV
Date: 02-2012
DOI: 10.1016/J.PMR.2011.11.015
Abstract: This article provides an overview of the structure and function of the National Skeletal Muscle Research Center (NSMRC) at the University of California, San Diego, which is one of the 7 research centers of the Medical Rehabilitation Research Infrastructure Network, created to facilitate access for physicians to experts, technology, and resources from scientific fields related to medical rehabilitation. The 4 cores of the NSMRC are described as a resource for rehabilitation medicine practitioners to use for clinically relevant muscle research.
Publisher: Springer Science and Business Media LLC
Date: 07-01-2016
DOI: 10.1038/NRDP.2015.82
Publisher: The Company of Biologists
Date: 2013
DOI: 10.1242/JCS.137026
Abstract: Nemaline myopathy (NM) is a congenital myopathy with an estimated incidence of 1∶50.000 live births, caused by mutations in thin filament components, including nebulin, accounting for about 50% of the cases. The identification of NM cases with nonsense mutations resulting in loss of the extreme C-terminal SH3 domain of nebulin suggests an important role of the nebulin SH3 domain, which is further supported by the recent demonstration of its role in IGF-1-induced sarcomeric actin filament formation through targeting of N-WASP to the Z-line. To provide further insights into the functional significance of the nebulin SH3 domain in the Z-disk and understand the mechanisms by which truncations of nebulin lead to NM, we took two approaches: 1) an affinity-based proteomic screening to identify novel interaction partners of the nebulin SH3 domain and 2) generation and characterization of a novel knock-in mouse model with a premature stop codon in the nebulin gene, eliminating its C-terminal SH3 domain (NebΔSH3 mouse). Surprisingly, detailed analyses of NebΔSH3 mice revealed no structural or histological skeletal muscle abnormalities and no changes in gene expression or localization of interaction partners of the nebulin SH3 domain, including myopalladin, palladin, zyxin, and N-WASP. Also, no significant effect on peak isometric stress production, passive tensile stress, or Young's modulus was found. However, NebΔSH3 muscle displayed a slightly altered force-frequency relationship and was significantly more susceptible to eccentric contraction-induced injury, suggesting that the nebulin SH3 domain plays a role in bearing high active stress and perhaps in fine-tuning the excitation-contraction coupling mechanism.
Publisher: Elsevier BV
Date: 05-2017
Publisher: Wiley
Date: 26-09-2016
DOI: 10.1002/MUS.25379
Publisher: American Physiological Society
Date: 15-04-2013
DOI: 10.1152/PHYSIOLGENOMICS.00132.2012
Abstract: In this work, the interaction between the loss of a primary component of the skeletal muscle cytoskeleton, desmin, and two common physiological stressors, acute mechanical injury and aging, were investigated at the transcriptional, protein, and whole muscle levels. The transcriptional response of desmin knockout ( des −/− ) plantarflexors to a bout of 50 eccentric contractions (ECCs) showed substantial overlap with the response in wild-type ( wt) muscle. However, changes in the expression of genes involved in muscle response to injury were blunted in adult des −/− muscle compared with wt (fold change with ECC in des −/− and wt, respectively: Mybph, 1.4 and 2.9 Xirp1, 2.2 and 5.7 Csrp3, 1.8 and 4.3), similar to the observed blunted mechanical response (torque drop: des −/− 30.3% and wt 55.5%). Interestingly, in the absence of stressors, des −/− muscle exhibited elevated expression of many these genes compared with wt. The largest transcriptional changes were observed in the interaction between aging and the absence of desmin, including many genes related to slow fiber pathway (Myh7, Myl3, Atp2a2, and Casq2) and insulin sensitivity (Tlr4, Trib3, Pdk3, and Pdk4). Consistent with these transcriptional changes, adult des −/− muscle exhibited a significant fiber type shift from fast to slow isoforms of myosin heavy chain ( wt, 5.3% IIa and 71.7% IIb des −/− , 8.4% IIa and 61.4% IIb) and a decreased insulin-stimulated glucose uptake ( wt, 0.188 μmol/g muscle/20 min des −/− , 0.085 μmol/g muscle/20 min). This work points to novel areas of influence of this cytoskeletal protein and directs future work to elucidate its function.
Publisher: Elsevier BV
Date: 08-2017
Publisher: Elsevier BV
Date: 2010
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 17-09-2014
DOI: 10.2106/JBJS.M.01585
Location: United States of America
Location: United States of America
No related grants have been discovered for Richard Lieber.