Literature DB >> 31917998

CLPB (caseinolytic peptidase B homolog), the first mitochondrial protein refoldase associated with human disease.

Dagmara Mróz1, Hubert Wyszkowski2, Tomasz Szablewski3, Katarzyna Zawieracz3, Rafał Dutkiewicz4, Katarzyna Bury5, Saskia B Wortmann6, Ron A Wevers7, Szymon Ziętkiewicz8.   

Abstract

Entities:  

Keywords:  AAA+ ATPase; Chaperone; Disaggregase; HSP100; Mitochondrial disease; Neutropenia

Year:  2020        PMID： 31917998     DOI： 10.1016/j.bbagen.2020.129512

Source DB:  PubMed          Journal:  Biochim Biophys Acta Gen Subj        ISSN： 0304-4165            Impact factor:   3.770


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  5 in total

1.  Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3.

Authors:  Ryan R Cupo; Alexandrea N Rizo; Gabriel A Braun; Eric Tse; Edward Chuang; Kushol Gupta; Daniel R Southworth; James Shorter
Journal:  Cell Rep       Date:  2022-09-27       Impact factor: 9.995

2.  Expression and Purification of Recombinant Skd3 (Human ClpB) Protein and Tobacco Etch Virus (TEV) Protease from Escherichia coli.

Authors:  Ryan R Cupo; James Shorter
Journal:  Bio Protoc       Date:  2020-12-05

3.  Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations.

Authors:  Ryan R Cupo; James Shorter
Journal:  Elife       Date:  2020-06-23       Impact factor: 8.140

4.  Heterozygous variants of CLPB are a cause of severe congenital neutropenia.

Authors:  Julia T Warren; Ryan R Cupo; Peeradol Wattanasirakul; David H Spencer; Adam E Locke; Vahagn Makaryan; Audrey Anna Bolyard; Merideth L Kelley; Natalie L Kingston; James Shorter; Christine Bellanné-Chantelot; Jean Donadieu; David C Dale; Daniel C Link
Journal:  Blood       Date:  2022-02-03       Impact factor: 25.476

Review 5.  Mitochondrial Protein Homeostasis and Cardiomyopathy.

Authors:  Emily Wachoski-Dark; Tian Zhao; Aneal Khan; Timothy E Shutt; Steven C Greenway
Journal:  Int J Mol Sci       Date:  2022-03-20       Impact factor: 5.923
  5 in total

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