title: Defective Ribonucleoproteins, Mistakes in RNA Processing, and Diseases.
abstract: Ribonucleoproteins (RNPs) are vital to many cellular events. To this end, many neurodegenerative diseases and cancers have been linked to RNP malfunction, particularly as this relates to defective processing of cellular RNA. The connection of RNPs and diseases has also propagated a shift of focus onto RNA targeting from traditional protein targeting treatments. However, therapeutic development in this area has been limited by incomplete molecular insight into the specific contributions of RNPs to disease. This review outlines the role of several RNPs in diseases, focusing on molecular defects in processes that affect proper RNA handling in the cell. This work also evaluates the contributions of recently developed methods to understanding RNP association and function. We review progress in this area by focusing on molecular malfunctions of RNPs associated with the onset and progression of several neurodegenerative diseases and cancer and conclude with a brief discussion of RNA-based therapeutic efforts.
authors.affiliation: McKetta Department of Chemical Engineering, University of Texas at Austin , 200 East. Dean Keeton Street, Stop C0400, Austin, Texas 78712, United States.
authors.forename: Mia K
authors.initials: MK
authors.lastname: Mihailovic
authors.affiliation: McKetta Department of Chemical Engineering, University of Texas at Austin , 200 East. Dean Keeton Street, Stop C0400, Austin, Texas 78712, United States.
authors.forename: Angela
authors.initials: A
authors.lastname: Chen
authors.affiliation: McKetta Department of Chemical Engineering, University of Texas at Austin , 200 East. Dean Keeton Street, Stop C0400, Austin, Texas 78712, United States.
authors.forename: Juan C
authors.initials: JC
authors.lastname: Gonzalez-Rivera
authors.affiliation: McKetta Department of Chemical Engineering, University of Texas at Austin , 200 East. Dean Keeton Street, Stop C0400, Austin, Texas 78712, United States.
authors.forename: Lydia M
authors.identifier: http://orcid.org/0000-0001-5010-5511
authors.initials: LM
authors.lastname: Contreras
mesh_terms: D000544:Alzheimer Disease / Q000235:genetics* / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics* / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D020868:Gene Silencing; D006801:Humans; D009134:Muscular Atrophy, Spinal / Q000235:genetics* / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D009154:Mutation; D009369:Neoplasms / Q000737:chemistry / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D016376:Oligonucleotides, Antisense / Q000627:therapeutic use; D066263:Protein Aggregation, Pathological / Q000235:genetics* / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D014176:Protein Biosynthesis; D012313:RNA / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D012323:RNA Processing, Post-Transcriptional; D012326:RNA Splicing; D034741:RNA, Small Interfering / Q000627:therapeutic use; D012261:Ribonucleoproteins / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism
chemical_list: D016376:Oligonucleotides, Antisense; D034741:RNA, Small Interfering; D012261:Ribonucleoproteins; D012313:RNA