{"type": "root", "children": [{"type": "p", "children": [{"type": "t", "text": "\nThe collected studies emphasize the importance of proper protein assembly and turnover for muscle integrity and function. For instance, Kelch family proteins have emerged as crucial regulators that prevent aggregation and degradation of key sarcomeric components. KLHL41, in particular, acts as a molecular chaperone that stabilizes nebulin through a unique poly-ubiquitination mechanism, thus safeguarding sarcomere organization and muscle contractility."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": " Similarly, sarcosin (also called Krp1) has been shown to be central to myofibrillogenesis by promoting the lateral fusion of nascent thin fibrils, ensuring that mature myofibrils are correctly assembled."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "2"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nFurther investigations reveal that the proper ubiquitination of sarcomeric regulators is critical for myofibril maturation. In one study, aberrant regulation of the thin filament chaperone NRAP by KLHL41 was linked to the pathogenesis of nemaline myopathy, highlighting that defects in ubiquitination pathways can lead to the improper accumulation or degradation of essential muscle proteins."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "3"}]}, {"type": "t", "text": " Additionally, temporal and spatial expression patterns of sarcosin during skeletal muscle development underscore its dual role: while its reduction in early differentiation may enhance myogenic progression, it becomes crucial at later stages to support the structural integrity of the developing myofibrils."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "4"}]}, {"type": "t", "text": "\n"}]}, {"type": "t", "text": "\n\n"}, {"type": "p", "children": [{"type": "t", "text": "\nIt is important to note that although the inquiry specifically mentions SNORD114-26, none of the referenced abstracts provide any information on the function or role of SNORD114-26. Instead, these studies collectively define mechanisms by which Kelch proteins modulate sarcomere assembly and muscle function, leaving the activity and biological significance of SNORD114-26 in this context undetermined.\n"}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Andres Ramirez-Martinez, Bercin Kutluk Cenik, Svetlana Bezprozvannaya, et al. "}, {"type": "b", "children": [{"type": "t", "text": "KLHL41 stabilizes skeletal muscle sarcomeres by nonproteolytic ubiquitination."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Elife (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.7554/eLife.26439"}], "href": "https://doi.org/10.7554/eLife.26439"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "28826497"}], "href": "https://pubmed.ncbi.nlm.nih.gov/28826497"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Cynthia C Greenberg, Patricia S Connelly, Mathew P Daniels, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Krp1 (Sarcosin) promotes lateral fusion of myofibril assembly intermediates in cultured mouse cardiomyocytes."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Exp Cell Res (2008)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.yexcr.2007.12.009"}], "href": "https://doi.org/10.1016/j.yexcr.2007.12.009"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "18178185"}], "href": "https://pubmed.ncbi.nlm.nih.gov/18178185"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Caroline Jirka, Jasmine H Pak, Claire A Grosgogeat, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Dysregulation of NRAP degradation by KLHL41 contributes to pathophysiology in nemaline myopathy."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hum Mol Genet (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1093/hmg/ddz078"}], "href": "https://doi.org/10.1093/hmg/ddz078"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "30986853"}], "href": "https://pubmed.ncbi.nlm.nih.gov/30986853"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Leonie du Puy, Abdelaziz Beqqali, Helena T A van Tol, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Sarcosin (Krp1) in skeletal muscle differentiation: gene expression profiling and knockdown experiments."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Int J Dev Biol (2012)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1387/ijdb.113327lp"}], "href": "https://doi.org/10.1387/ijdb.113327lp"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "22562206"}], "href": "https://pubmed.ncbi.nlm.nih.gov/22562206"}]}]}]}