Mitochondrial protein import and human health and disease

James A. MacKenzie, R. Payne

Research output: Contribution to journalArticle

69 Citations (Scopus)

Abstract

The targeting and assembly of nuclear-encoded mitochondrial proteins are essential processes because the energy supply of humans is dependent upon the proper functioning of mitochondria. Defective import of mitochondrial proteins can arise from mutations in the targeting signals within precursor proteins, from mutations that disrupt the proper functioning of the import machinery, or from deficiencies in the chaperones involved in the proper folding and assembly of proteins once they are imported. Defects in these steps of import have been shown to lead to oxidative stress, neurodegenerative diseases, and metabolic disorders. In addition, protein import into mitochondria has been found to be a dynamically regulated process that varies in response to conditions such as oxidative stress, aging, drug treatment, and exercise. This review focuses on how mitochondrial protein import affects human health and disease.

Original languageEnglish
Pages (from-to)509-523
Number of pages15
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1772
Issue number5
DOIs
StatePublished - May 2007

Fingerprint

Mitochondrial Proteins
Health
Mitochondria
Oxidative Stress
Mutation
Protein Precursors
Protein Folding
Neurodegenerative Diseases
Pharmaceutical Preparations
Proteins

Keywords

  • Human health and disease
  • Mitochondria
  • Protein targeting
  • Protein translocation

ASJC Scopus subject areas

  • Molecular Biology
  • Molecular Medicine
  • Biophysics

Cite this

Mitochondrial protein import and human health and disease. / MacKenzie, James A.; Payne, R.

In: Biochimica et Biophysica Acta - Molecular Basis of Disease, Vol. 1772, No. 5, 05.2007, p. 509-523.

Research output: Contribution to journalArticle

@article{8f0fc0792cb5436eb2d7a5ac8f045355,
title = "Mitochondrial protein import and human health and disease",
abstract = "The targeting and assembly of nuclear-encoded mitochondrial proteins are essential processes because the energy supply of humans is dependent upon the proper functioning of mitochondria. Defective import of mitochondrial proteins can arise from mutations in the targeting signals within precursor proteins, from mutations that disrupt the proper functioning of the import machinery, or from deficiencies in the chaperones involved in the proper folding and assembly of proteins once they are imported. Defects in these steps of import have been shown to lead to oxidative stress, neurodegenerative diseases, and metabolic disorders. In addition, protein import into mitochondria has been found to be a dynamically regulated process that varies in response to conditions such as oxidative stress, aging, drug treatment, and exercise. This review focuses on how mitochondrial protein import affects human health and disease.",
keywords = "Human health and disease, Mitochondria, Protein targeting, Protein translocation",
author = "MacKenzie, {James A.} and R. Payne",
year = "2007",
month = "5",
doi = "10.1016/j.bbadis.2006.12.002",
language = "English",
volume = "1772",
pages = "509--523",
journal = "Biochimica et Biophysica Acta - Molecular Basis of Disease",
issn = "0925-4439",
publisher = "Elsevier",
number = "5",

}

TY - JOUR

T1 - Mitochondrial protein import and human health and disease

AU - MacKenzie, James A.

AU - Payne, R.

PY - 2007/5

Y1 - 2007/5

N2 - The targeting and assembly of nuclear-encoded mitochondrial proteins are essential processes because the energy supply of humans is dependent upon the proper functioning of mitochondria. Defective import of mitochondrial proteins can arise from mutations in the targeting signals within precursor proteins, from mutations that disrupt the proper functioning of the import machinery, or from deficiencies in the chaperones involved in the proper folding and assembly of proteins once they are imported. Defects in these steps of import have been shown to lead to oxidative stress, neurodegenerative diseases, and metabolic disorders. In addition, protein import into mitochondria has been found to be a dynamically regulated process that varies in response to conditions such as oxidative stress, aging, drug treatment, and exercise. This review focuses on how mitochondrial protein import affects human health and disease.

AB - The targeting and assembly of nuclear-encoded mitochondrial proteins are essential processes because the energy supply of humans is dependent upon the proper functioning of mitochondria. Defective import of mitochondrial proteins can arise from mutations in the targeting signals within precursor proteins, from mutations that disrupt the proper functioning of the import machinery, or from deficiencies in the chaperones involved in the proper folding and assembly of proteins once they are imported. Defects in these steps of import have been shown to lead to oxidative stress, neurodegenerative diseases, and metabolic disorders. In addition, protein import into mitochondria has been found to be a dynamically regulated process that varies in response to conditions such as oxidative stress, aging, drug treatment, and exercise. This review focuses on how mitochondrial protein import affects human health and disease.

KW - Human health and disease

KW - Mitochondria

KW - Protein targeting

KW - Protein translocation

UR - http://www.scopus.com/inward/record.url?scp=34247127747&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=34247127747&partnerID=8YFLogxK

U2 - 10.1016/j.bbadis.2006.12.002

DO - 10.1016/j.bbadis.2006.12.002

M3 - Article

C2 - 17300922

AN - SCOPUS:34247127747

VL - 1772

SP - 509

EP - 523

JO - Biochimica et Biophysica Acta - Molecular Basis of Disease

JF - Biochimica et Biophysica Acta - Molecular Basis of Disease

SN - 0925-4439

IS - 5

ER -