TMAO to the rescue of pathogenic protein variants

Trimethylamine N-oxide (TMAO) is a chemical chaperone found in various organisms including humans. Various studies unveiled that it is an excellent protein-stabilizing agent, and induces folding of unstructured proteins. It is also well established that it can counteract the deleterious effects of u...

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Published inBiochimica et biophysica acta. General subjects Vol. 1866; no. 11; p. 130214
Main Authors Kumari, Kritika, Singh, Khuraijam Surjalal, Singh, Kuldeep, Bakhshi, Radhika, Singh, Laishram Rajendrakumar
Format Journal Article
LanguageEnglish
Published Elsevier B.V 01.11.2022
Subjects
Chemical chaperone
Functional rescue
human diseases
hydrostatic pressure
Mutant protein
mutants
Protein conformational diseases
protein folding
protein subunits
Proteopathies
therapeutics
toxicity
trimethylamine
urea
Chemical chaperone
Proteopathies
Mutant protein
Protein conformational diseases
Functional rescue
Online AccessGet full text
ISSN0304-4165
1872-8006
1872-8006
DOI10.1016/j.bbagen.2022.130214

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Abstract Trimethylamine N-oxide (TMAO) is a chemical chaperone found in various organisms including humans. Various studies unveiled that it is an excellent protein-stabilizing agent, and induces folding of unstructured proteins. It is also well established that it can counteract the deleterious effects of urea, salt, and hydrostatic pressure on macromolecular integrity. There is also existence of large body of data regarding its ability to restore functional deficiency of various mutant proteins or pathogenic variants by correcting misfolding defects and inhibiting the formation of high-order toxic protein oligomers. Since an important class of human disease called “protein conformational disorders” is due to protein misfolding and/or formation of high-order oligomers, TMAO stands as a promising molecule for the therapeutic intervention of such diseases. The present review has been designed to gather a comprehensive knowledge of the TMAO's effect on the functional restoration of various mutants, identify its shortcomings and explore its potentiality as a lead molecule. Future prospects have also been suitably incorporated. •TMAO could restore function to the function to temperature sensitive mutant proteins.•TMAO inhibits formation of toxic high-order oligomers.•TMAO could prevent ERAD-mediated degradation of mutant proteins and help to correct trafficking defect.•TMAO is a potential molecule that can be used for therapeutic interventions of protein conformational disorders.
AbstractList Trimethylamine N-oxide (TMAO) is a chemical chaperone found in various organisms including humans. Various studies unveiled that it is an excellent protein-stabilizing agent, and induces folding of unstructured proteins. It is also well established that it can counteract the deleterious effects of urea, salt, and hydrostatic pressure on macromolecular integrity. There is also existence of large body of data regarding its ability to restore functional deficiency of various mutant proteins or pathogenic variants by correcting misfolding defects and inhibiting the formation of high-order toxic protein oligomers. Since an important class of human disease called “protein conformational disorders” is due to protein misfolding and/or formation of high-order oligomers, TMAO stands as a promising molecule for the therapeutic intervention of such diseases. The present review has been designed to gather a comprehensive knowledge of the TMAO's effect on the functional restoration of various mutants, identify its shortcomings and explore its potentiality as a lead molecule. Future prospects have also been suitably incorporated.
Trimethylamine N-oxide (TMAO) is a chemical chaperone found in various organisms including humans. Various studies unveiled that it is an excellent protein-stabilizing agent, and induces folding of unstructured proteins. It is also well established that it can counteract the deleterious effects of urea, salt, and hydrostatic pressure on macromolecular integrity. There is also existence of large body of data regarding its ability to restore functional deficiency of various mutant proteins or pathogenic variants by correcting misfolding defects and inhibiting the formation of high-order toxic protein oligomers. Since an important class of human disease called "protein conformational disorders" is due to protein misfolding and/or formation of high-order oligomers, TMAO stands as a promising molecule for the therapeutic intervention of such diseases. The present review has been designed to gather a comprehensive knowledge of the TMAO's effect on the functional restoration of various mutants, identify its shortcomings and explore its potentiality as a lead molecule. Future prospects have also been suitably incorporated.Trimethylamine N-oxide (TMAO) is a chemical chaperone found in various organisms including humans. Various studies unveiled that it is an excellent protein-stabilizing agent, and induces folding of unstructured proteins. It is also well established that it can counteract the deleterious effects of urea, salt, and hydrostatic pressure on macromolecular integrity. There is also existence of large body of data regarding its ability to restore functional deficiency of various mutant proteins or pathogenic variants by correcting misfolding defects and inhibiting the formation of high-order toxic protein oligomers. Since an important class of human disease called "protein conformational disorders" is due to protein misfolding and/or formation of high-order oligomers, TMAO stands as a promising molecule for the therapeutic intervention of such diseases. The present review has been designed to gather a comprehensive knowledge of the TMAO's effect on the functional restoration of various mutants, identify its shortcomings and explore its potentiality as a lead molecule. Future prospects have also been suitably incorporated.
Trimethylamine N-oxide (TMAO) is a chemical chaperone found in various organisms including humans. Various studies unveiled that it is an excellent protein-stabilizing agent, and induces folding of unstructured proteins. It is also well established that it can counteract the deleterious effects of urea, salt, and hydrostatic pressure on macromolecular integrity. There is also existence of large body of data regarding its ability to restore functional deficiency of various mutant proteins or pathogenic variants by correcting misfolding defects and inhibiting the formation of high-order toxic protein oligomers. Since an important class of human disease called “protein conformational disorders” is due to protein misfolding and/or formation of high-order oligomers, TMAO stands as a promising molecule for the therapeutic intervention of such diseases. The present review has been designed to gather a comprehensive knowledge of the TMAO's effect on the functional restoration of various mutants, identify its shortcomings and explore its potentiality as a lead molecule. Future prospects have also been suitably incorporated. •TMAO could restore function to the function to temperature sensitive mutant proteins.•TMAO inhibits formation of toxic high-order oligomers.•TMAO could prevent ERAD-mediated degradation of mutant proteins and help to correct trafficking defect.•TMAO is a potential molecule that can be used for therapeutic interventions of protein conformational disorders.
ArticleNumber 130214
Author Singh, Khuraijam Surjalal
Bakhshi, Radhika
Singh, Kuldeep
Singh, Laishram Rajendrakumar
Kumari, Kritika
Author_xml – sequence: 1
  givenname: Kritika
  surname: Kumari
  fullname: Kumari, Kritika
  organization: Dr. B. R. Ambedkar Center for Biomedical Research, University of Delhi, Delhi 110007, India
– sequence: 2
  givenname: Khuraijam Surjalal
  surname: Singh
  fullname: Singh, Khuraijam Surjalal
  organization: Dr. B. R. Ambedkar Center for Biomedical Research, University of Delhi, Delhi 110007, India
– sequence: 3
  givenname: Kuldeep
  surname: Singh
  fullname: Singh, Kuldeep
  organization: Dr. B. R. Ambedkar Center for Biomedical Research, University of Delhi, Delhi 110007, India
– sequence: 4
  givenname: Radhika
  surname: Bakhshi
  fullname: Bakhshi, Radhika
  organization: Department of Biomedical Sciences, Shaheed Rajguru College of Applied Sciences for Women, University of Delhi, Delhi 110096, India
– sequence: 5
  givenname: Laishram Rajendrakumar
  surname: Singh
  fullname: Singh, Laishram Rajendrakumar
  email: lairksingh@gmail.com
  organization: Dr. B. R. Ambedkar Center for Biomedical Research, University of Delhi, Delhi 110007, India
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Keywords Chemical chaperone
Proteopathies
Mutant protein
Protein conformational diseases
Functional rescue
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Snippet Trimethylamine N-oxide (TMAO) is a chemical chaperone found in various organisms including humans. Various studies unveiled that it is an excellent...
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SubjectTerms Chemical chaperone
Functional rescue
human diseases
hydrostatic pressure
Mutant protein
mutants
Protein conformational diseases
protein folding
protein subunits
Proteopathies
therapeutics
toxicity
trimethylamine
urea
Title TMAO to the rescue of pathogenic protein variants
URI https://dx.doi.org/10.1016/j.bbagen.2022.130214
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