A review: Molecular chaperone-mediated folding, unfolding and disaggregation of expressed recombinant proteins
K Fatima, F Naqvi, H Younas - Cell Biochemistry and Biophysics, 2021 - Springer
The advancements in biotechnology over time have led to an increase in the demand of
pure, soluble and functionally active proteins. Recombinant protein production has thus …
pure, soluble and functionally active proteins. Recombinant protein production has thus …
[HTML][HTML] Mitochondrial protein homeostasis and cardiomyopathy
Human mitochondrial disorders impact tissues with high energetic demands and can be
associated with cardiac muscle disease (cardiomyopathy) and early mortality. However, the …
associated with cardiac muscle disease (cardiomyopathy) and early mortality. However, the …
[HTML][HTML] Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations
Cells have evolved specialized protein disaggregases to reverse toxic protein aggregation
and restore protein functionality. In nonmetazoan eukaryotes, the AAA+ disaggregase …
and restore protein functionality. In nonmetazoan eukaryotes, the AAA+ disaggregase …
Heterozygous variants of CLPB are a cause of severe congenital neutropenia
Severe congenital neutropenia is an inborn disorder of granulopoiesis. Approximately one
third of cases do not have a known genetic cause. Exome sequencing of 104 persons with …
third of cases do not have a known genetic cause. Exome sequencing of 104 persons with …
Neutropenia and intellectual disability are hallmarks of biallelic and de novo CLPB deficiency
SB Wortmann, S Ziętkiewicz, S Guerrero-Castillo… - Genetics in …, 2021 - nature.com
Purpose To investigate monoallelic CLPB variants. Pathogenic variants in many genes
cause congenital neutropenia. While most patients exhibit isolated hematological …
cause congenital neutropenia. While most patients exhibit isolated hematological …
[HTML][HTML] Structural basis of impaired disaggregase function in the oxidation-sensitive SKD3 mutant causing 3-methylglutaconic aciduria
Mitochondria are critical to cellular and organismal health. To prevent damage, mitochondria
have evolved protein quality control machines to survey and maintain the mitochondrial …
have evolved protein quality control machines to survey and maintain the mitochondrial …
[HTML][HTML] Comprehensive structural characterization of the human AAA+ disaggregase CLPB in the apo-and substrate-bound states reveals a unique mode of action …
D Wu, Y Liu, Y Dai, G Wang, G Lu, Y Chen, N Li… - PLoS …, 2023 - journals.plos.org
The human AAA+ ATPase CLPB (SKD3) is a protein disaggregase in the mitochondrial
intermembrane space (IMS) and functions to promote the solubilization of various …
intermembrane space (IMS) and functions to promote the solubilization of various …
[HTML][HTML] Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3
Summary The AAA+ protein, Skd3 (human CLPB), solubilizes proteins in the mitochondrial
intermembrane space, which is critical for human health. Skd3 variants with defective protein …
intermembrane space, which is critical for human health. Skd3 variants with defective protein …
Dodecamer assembly of a metazoan AAA+ chaperone couples substrate extraction to refolding
Ring-forming AAA+ chaperones solubilize protein aggregates and protect organisms from
proteostatic stress. In metazoans, the AAA+ chaperone Skd3 in the mitochondrial …
proteostatic stress. In metazoans, the AAA+ chaperone Skd3 in the mitochondrial …
[HTML][HTML] Therapeutic genetic variation revealed in diverse Hsp104 homologs
ZM March, K Sweeney, H Kim, X Yan, LM Castellano… - elife, 2020 - elifesciences.org
The AAA+ protein disaggregase, Hsp104, increases fitness under stress by reversing stress-
induced protein aggregation. Natural Hsp104 variants might exist with enhanced, selective …
induced protein aggregation. Natural Hsp104 variants might exist with enhanced, selective …