Results 41 to 50 of about 6,880,999 (291)

Colocalization of 14-3-3 proteins with SOD1 in Lewy body-like hyaline inclusions in familial amyotrophic lateral sclerosis cases and the animal model. [PDF]

open access: yesPLoS ONE, 2011
Cu/Zn superoxide dismutase (SOD1) is a major component of Lewy body-like hyaline inclusion (LBHI) found in the postmortem tissue of SOD1-linked familial amyotrophic lateral sclerosis (FALS) patients. In our recent studies, 14-3-3 proteins have been found
Yoko Okamoto   +12 more
doaj   +1 more source

Dorfin-CHIP chimeric proteins potently ubiquitylate and degrade familial ALS-related mutant SOD1 proteins and reduce their cellular toxicity

open access: yesNeurobiology of Disease, 2007
The ubiquitin–proteasome system (UPS) is involved in the pathogenetic mechanisms of neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS).
Shinsuke Ishigaki   +8 more
doaj   +1 more source

A Novel Approach to Recovery of Function of Mutant Proteins by Slowing Down Translation*

open access: yesJournal of Biological Chemistry, 2012
Background: Current strategies to alleviate protein misfolding include manipulation of chaperones, proteasomes, or autophagy. Results: Mild translation inhibition disproportionally blocked production of misfolded proteins and improved mutant CFTR ...
A. Meriin   +7 more
semanticscholar   +1 more source

Revealing the structure of land plant photosystem II: the journey from negative‐stain EM to cryo‐EM

open access: yesFEBS Letters, EarlyView.
Advances in cryo‐EM have revealed the detailed structure of Photosystem II, a key protein complex driving photosynthesis. This review traces the journey from early low‐resolution images to high‐resolution models, highlighting how these discoveries deepen our understanding of light harvesting and energy conversion in plants.
Roman Kouřil
wiley   +1 more source

Mutations in PIH proteins MOT48, TWI1 and PF13 define common and unique steps for preassembly of each, different ciliary dynein.

open access: yesPLoS Genetics, 2020
Ciliary dyneins are preassembled in the cytoplasm before being transported into cilia, and a family of proteins containing the PIH1 domain, PIH proteins, are involved in the assembly process.
Ryosuke Yamamoto   +7 more
doaj   +1 more source

Defective Proteasome Delivery of Polyubiquitinated Proteins by Ubiquilin-2 Proteins Containing ALS Mutations. [PDF]

open access: yesPLoS ONE, 2015
Ubiquilin proteins facilitate delivery of ubiquitinated proteins to the proteasome for degradation. Interest in the proteins has been heightened by the discovery that gene mutations in UBQLN2 cause dominant inheritance of amyotrophic lateral sclerosis ...
Lydia Chang, Mervyn J Monteiro
doaj   +1 more source

Mutant p53 as a guardian of the cancer cell

open access: yesCell Death and Differentiation, 2018
Forty years of research have established that the p53 tumor suppressor provides a major barrier to neoplastic transformation and tumor progression by its unique ability to act as an extremely sensitive collector of stress inputs, and to coordinate a ...
F. Mantovani, Licio Collavin, G. Del Sal
semanticscholar   +1 more source

Mapping the evolution of mitochondrial complex I through structural variation

open access: yesFEBS Letters, EarlyView.
Respiratory complex I (CI) is crucial for bioenergetic metabolism in many prokaryotes and eukaryotes. It is composed of a conserved set of core subunits and additional accessory subunits that vary depending on the organism. Here, we categorize CI subunits from available structures to map the evolution of CI across eukaryotes. Respiratory complex I (CI)
Dong‐Woo Shin   +2 more
wiley   +1 more source

The mutation V42M distorts the compact packing of the human gamma-S-crystallin molecule, resulting in congenital cataract. [PDF]

open access: yesPLoS ONE, 2012
BACKGROUND: Human γS-crystallin is an important component of the human eye lens nucleus and cortex. The mutation V42M in the molecule causes severe congenital cataract in children. We compare the structure of the mutant protein with that of the wild type
Venkata Pulla Rao Vendra   +2 more
doaj   +1 more source

Genome-wide Analysis of Transcription Factor E2F1 Mutant Proteins Reveals That N- and C-terminal Protein Interaction Domains Do Not Participate in Targeting E2F1 to the Human Genome

open access: yesJournal of Biological Chemistry, 2011
Previous studies of E2F family members have suggested that protein-protein interactions may be the mechanism by which E2F proteins are recruited to specific genomic regions. We have addressed this hypothesis on a genome-wide scale using ChIP-seq analysis
Alina R. Cao   +5 more
semanticscholar   +1 more source

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