Cargando…

Palmitoylation-induced Aggregation of Cysteine-string Protein Mutants That Cause Neuronal Ceroid Lipofuscinosis

Recently, mutations in the DNAJC5 gene encoding cysteine-string protein α (CSPα) were identified to cause the neurodegenerative disorder adult-onset neuronal ceroid lipofuscinosis. The disease-causing mutations (L115R or ΔL116) occur within the cysteine-string domain, a region of the protein that is...

Descripción completa

Detalles Bibliográficos
Autores principales:	Greaves, Jennifer, Lemonidis, Kimon, Gorleku, Oforiwa A., Cruchaga, Carlos, Grefen, Christopher, Chamberlain, Luke H.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	American Society for Biochemistry and Molecular Biology 2012
Materias:	Protein Structure and Folding
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3481330/ https://www.ncbi.nlm.nih.gov/pubmed/22902780 http://dx.doi.org/10.1074/jbc.M112.389098

Ejemplares similares

A cluster of palmitoylated cysteines are essential for aggregation of cysteine-string protein mutants that cause neuronal ceroid lipofuscinosis
por: Diez-Ardanuy, Cinta, et al.
Publicado: (2017)

Crystal Structure and Autoactivation Pathway of the Precursor Form of Human Tripeptidyl-peptidase 1, the Enzyme Deficient in Late Infantile Ceroid Lipofuscinosis
por: Guhaniyogi, Jayita, et al.
Publicado: (2009)

Mutant p53 Aggregates into Prion-like Amyloid Oligomers and Fibrils: IMPLICATIONS FOR CANCER
por: Ano Bom, Ana P. D., et al.
Publicado: (2012)

The Golgi S-acylation machinery comprises zDHHC enzymes with major differences in substrate affinity and S-acylation activity
por: Lemonidis, Kimon, et al.
Publicado: (2014)

Iron-mediated Aggregation and a Localized Structural Change Characterize Ferritin from a Mutant Light Chain Polypeptide That Causes Neurodegeneration
por: Baraibar, Martin A., et al.
Publicado: (2008)

N-helix and Cysteines Inter-regulate Human Mitochondrial VDAC-2 Function and Biochemistry
por: Maurya, Svetlana Rajkumar, et al.
Publicado: (2015)

C-terminal α-synuclein truncations are linked to cysteine cathepsin activity in Parkinson's disease
por: McGlinchey, Ryan P., et al.
Publicado: (2019)

Aggregation-primed molten globule conformers of the p53 core domain provide potential tools for studying p53C aggregation in cancer
por: Pedrote, Murilo M., et al.
Publicado: (2018)

Stages and Conformations of the Tau Repeat Domain during Aggregation and Its Effect on Neuronal Toxicity
por: Kumar, Satish, et al.
Publicado: (2014)

Single-residue physicochemical characteristics kinetically partition membrane protein self-assembly and aggregation
por: Gupta, Ankit, et al.
Publicado: (2020)

Using Intramolecular Disulfide Bonds in Tau Protein to Deduce Structural Features of Aggregation-resistant Conformations
por: Walker, Sophie, et al.
Publicado: (2012)

Protein Aggregates Are Recruited to Aggresome by Histone Deacetylase 6 via Unanchored Ubiquitin C Termini
por: Ouyang, Hui, et al.
Publicado: (2012)

Structural transitions in Orb2 prion-like domain relevant for functional aggregation in memory consolidation
por: Oroz, Javier, et al.
Publicado: (2021)

Increased Monomerization of Mutant HSPB1 Leads to Protein Hyperactivity in Charcot-Marie-Tooth Neuropathy
por: Almeida-Souza, Leonardo, et al.
Publicado: (2010)

Methionine Oxidation of Sup35 Protein Induces Formation of the [PSI(+)] Prion in a Yeast Peroxiredoxin Mutant
por: Sideri, Theodora C., et al.
Publicado: (2011)

The Truncated C-terminal RNA Recognition Motif of TDP-43 Protein Plays a Key Role in Forming Proteinaceous Aggregates
por: Wang, Yi-Ting, et al.
Publicado: (2013)

Human Mitochondrial Chaperone (mtHSP70) and Cysteine Desulfurase (NFS1) Bind Preferentially to the Disordered Conformation, Whereas Co-chaperone (HSC20) Binds to the Structured Conformation of the Iron-Sulfur Cluster Scaffold Protein (ISCU)
por: Cai, Kai, et al.
Publicado: (2013)

Loss of the p53 transactivation domain results in high amyloid aggregation of the Δ40p53 isoform in endometrial carcinoma cells
por: Melo dos Santos, Nataly, et al.
Publicado: (2019)

A Platform to View Huntingtin Exon 1 Aggregation Flux in the Cell Reveals Divergent Influences from Chaperones hsp40 and hsp70
por: Ormsby, Angelique R., et al.
Publicado: (2013)

Prion Protein Complexed to N2a Cellular RNAs through Its N-terminal Domain Forms Aggregates and Is Toxic to Murine Neuroblastoma Cells
por: Gomes, Mariana P. B., et al.
Publicado: (2008)

Structure of the N-terminal Region of Complement Factor H and Conformational Implications of Disease-linked Sequence Variations
por: Hocking, Henry G., et al.
Publicado: (2008)

Crystal Structure of a Functional Dimer of the PhoQ Sensor Domain
por: Cheung, Jonah, et al.
Publicado: (2008)

Function and Structure of a Prokaryotic Formylglycine-generating Enzyme
por: Carlson, Brian L., et al.
Publicado: (2008)

Intra- and Intermonomer Interactions Are Required to Synergistically Facilitate ATP Hydrolysis in Hsp90
por: Cunningham, Christian N., et al.
Publicado: (2008)

A Common Theme in Interaction of Bacterial Immunoglobulin-binding Proteins with Immunoglobulins Illustrated in the Equine System
por: Lewis, Melanie J., et al.
Publicado: (2008)

A Common β-Sheet Architecture Underlies in Vitro and in Vivo β(2)-Microglobulin Amyloid Fibrils
por: Jahn, Thomas R., et al.
Publicado: (2008)

Appearance and Propagation of Polyglutamine-based Amyloids in Yeast: TYROSINE RESIDUES ENABLE POLYMER FRAGMENTATION
por: Alexandrov, Ilya M., et al.
Publicado: (2008)

Structural Consequences of Nucleophosmin Mutations in Acute Myeloid Leukemia
por: Grummitt, Charles G., et al.
Publicado: (2008)

Structure of the Human 26S Proteasome: SUBUNIT RADIAL DISPLACEMENTS OPEN THE GATE INTO THE PROTEOLYTIC CORE
por: da Fonseca, Paula C. A., et al.
Publicado: (2008)

Crystal Structure of Colicin M, a Novel Phosphatase Specifically Imported by Escherichia coli>
por: Zeth, Kornelius, et al.
Publicado: (2008)

Structure-Function Analysis of the C3 Binding Region of Staphylococcus aureus Immune Subversion Protein Sbi
por: Upadhyay, Abhishek, et al.
Publicado: (2008)

Structural Constraints for the Binding of Short Peptides to Claudin-4 Revealed by Surface Plasmon Resonance
por: Ling, Jun, et al.
Publicado: (2008)

Electrostatic and Lipid Anchor Contributions to the Interaction of Transducin with Membranes: MECHANISTIC IMPLICATIONS FOR ACTIVATION AND TRANSLOCATION
por: Kosloff, Mickey, et al.
Publicado: (2008)

Albumin-conjugated C34 Peptide HIV-1 Fusion Inhibitor: EQUIPOTENT TO C34 AND T-20 IN VITRO WITH SUSTAINED ACTIVITY IN SCID-HU THY/LIV MICE
por: Stoddart, Cheryl A., et al.
Publicado: (2008)

Deciphering the Ligand-binding Sites in the Borrelia burgdorferi Complement Regulator-acquiring Surface Protein 2 Required for Interactions with the Human Immune Regulators Factor H and Factor H-like Protein 1
por: Siegel, Corinna, et al.
Publicado: (2008)

Synaptotagmin C2B Domain Regulates Ca(2+)-triggered Fusion in Vitro: CRITICAL RESIDUES REVEALED BY SCANNING ALANINE MUTAGENESIS
por: Gaffaney, Jon D., et al.
Publicado: (2008)

Molecular Recognition of Chymotrypsin by the Serine Protease Inhibitor Ecotin from Yersinia pestis
por: Clark, Elizabeth A., et al.
Publicado: (2011)

Mapping the IκB Kinase β (IKKβ)-binding Interface of the B14 Protein, a Vaccinia Virus Inhibitor of IKKβ-mediated Activation of Nuclear Factor κB
por: Benfield, Camilla T. O., et al.
Publicado: (2011)

Structural and Functional Studies on the N-terminal Domain of the Shigella Type III Secretion Protein MxiG
por: McDowell, Melanie A., et al.
Publicado: (2011)

Truncation, Randomization, and Selection: GENERATION OF A REDUCED LENGTH c-Jun ANTAGONIST THAT RETAINS HIGH INTERACTION STABILITY
por: Crooks, Richard O., et al.
Publicado: (2011)

Cannot write session to /tmp/vufind_sessions/sess_mm3dahoqqd7ov1t4gkqt9mju0g