Point mutations within the CAP-gly domain of the p150(Glued) subunit of the dynactin complex have been identified in patients with distal spinal bulbar muscular atrophy (dSBMA) and Perry's syndrome. Herein, we show by CD and NMR experiments that each mutated CAP-gly domain is folded but less stable than the wild-type (WT) domain. We also demonstrate that the domains harboring these mutations bind to microtubules but fail to bind to EB1. These data indicate that these disease-associated, point mutations affect the stability of this domain and inhibit their interaction with EB1 but do not inhibit their interaction with microtubules.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2938955 | PMC |
| http://dx.doi.org/10.1021/bi100235z | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Hypothermic oxygenated perfusion inhibits CLIP1-mediated TIRAP ubiquitination via TFPI2 to reduce ischemia‒reperfusion injury of the fatty liver.
Exp Mol Med
December 2024
Zhongnan Hospital of Wuhan University, Institute of Hepatobiliary Diseases of Wuhan University, Transplant Center of Wuhan University, National Quality Control Center for Donated Organ Procurement, Hubei Key Laboratory of Medical Technology on Transplantation, Hubei Clinical Research Center for Natural Polymer Biological Liver, Hubei Engineering Center of Natural Polymer-based Medical Materials, 430071, Wuhan, China.
The use of fatty livers in liver transplantation has emerged as a crucial strategy to expand the pool of donor livers; however, fatty livers are more sensitive to ischemia‒reperfusion injury (IRI). Excessive congenital inflammatory responses are crucial in IRI. Hypothermic oxygenated perfusion (HOPE) is a novel organ preservation technique that may improve marginal donor liver quality by reducing the inflammatory response.
View Article and Find Full Text PDFFirst family with Perry syndrome from Mexico.
Biomed Rep
August 2024
Laboratory of Genomic Diagnostics, National Institute of Genomic Medicine, Mexico City 14610, Mexico.
Perry syndrome (PS) is a rare autosomal dominant disease characterized by parkinsonism, central hypoventilation, weight loss and depression and is caused by pathogenic mutations in the dynactin subunit 1 () gene (encoding p150 protein). To date, only two cases have been reported in Latin America, specifically in Colombia and Argentina. The present study, to the best of our knowledge, reports the first recorded Mexican family with PS.
View Article and Find Full Text PDFControl of motor landing and processivity by the CAP-Gly domain in the KIF13B tail.
Nat Commun
August 2023
Department of Molecular and Cellular Biology, University of California - Davis, 145 Briggs Hall, Davis, CA, 95616, USA.
Microtubules are major components of the eukaryotic cytoskeleton. Posttranslational modifications (PTMs) of tubulin regulates interactions with microtubule-associated proteins (MAPs). One unique PTM is the cyclical removal and re-addition of the C-terminal tyrosine of α-tubulin and MAPs containing CAP-Gly domains specifically recognize tyrosinated microtubules.
View Article and Find Full Text PDFBackground: Perry disease (or Perry syndrome [PS]) is a hereditary neurodegenerative disorder inevitably leading to death within few years from onset. All previous cases with pathological confirmation were caused by mutations within the cytoskeleton-associated protein glycine-rich (CAP-Gly) domain of the gene.
Objectives: This paper presents the first clinicopathological report of PS due to a novel mutation outside the CAP-Gly domain.
Carbon Dots from Attenuate Radiation-Induced Bone Injury by Inhibiting Senescence via METTL3/Clip3 in an mA-Dependent Manner.
ACS Appl Mater Interfaces
May 2023
Department of Oromaxillofacial-Head & Neck Oncology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, College of Stomatology, National Center for Stomatology, National Clinical Research Center for Oral Diseases, Shanghai Key Laboratory of Stomatology, Shanghai Research Institute of Stomatology, Shanghai Jiao Tong University, Shanghai 200011, China.
Radiation-induced bone injury management remains a challenge in clinical practice, and there is no effective medicine. Recently, biomass-derived carbon dots (CDs) have attracted attention in biomedical engineering due to the advantages of abundant heteroatoms, low toxicity, and no need to drug loading. Here, we report that CDs, synthesized from via hydrothermal strategy, can effectively alleviate radiation-induced bone injury.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!