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PMID: 3624306 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

The ornithine transcarbamylase leader peptide directs mitochondrial import through both its midportion structure and net positive charge.

The Journal of cell biology ·Vol. 105 ·No. 2 ·1987-08-00 ·Pages 669-77

Horwich AL, Kalousek F, Fenton WA, Furtak K, Pollock RA, Rosenberg LE

Abstract

The cytoplasmically synthesized precursor of the mitochondrial matrix enzyme, ornithine transcarbamylase (OTC), is targeted to mitochondria by its NH2-terminal leader peptide. We previously established through mutational analysis that the midportion of the OTC leader peptide is functionally required. In this article, we report that study of additional OTC precursors, altered in either a site-directed or random manner, reveals that (a) the midportion, but not the NH2-terminal half, is sufficient by itself to direct import, (b) the functional structure in the midportion is unlikely to be an amphiphilic alpha-helix, (c) the four arginines in the leader peptide contribute collectively to import function by conferring net positive charge, and (d) surprisingly, proteolytic processing of the leader peptide does not require the presence of a specific primary structure at the site of cleavage, in order to produce the mature OTC subunit.

MeSH Terms
Amino Acid Sequence Mitochondria/enzymology Mutation Ornithine Carbamoyltransferase/genetics Plasmids Protein Biosynthesis Protein Processing, Post-Translational Protein Sorting Signals/metabolism Transcription, Genetic
Chemicals
Protein Sorting Signals Ornithine Carbamoyltransferase
Authors & Affiliations
6 authors, click to expand affiliations / ORCID
Horwich A L
Kalousek F
Fenton W A
Furtak K
Pollock R A
Rosenberg L E
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20 references, click to expand
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Article Info
Journal
The Journal of cell biology
Abbr.
J Cell Biol
ISSN
0021-9525
DOI
10.1083/jcb.105.2.669
Published
1987-08-00
Pages
669-77
Language
English
Region
United States
NLM ID
0375356
PMCID
PMC2114782
Subset
IM
Grants
NIDDK NIH HHS · DK-09527 · United States
NIGMS NIH HHS · GM-32156 · United States
NIGMS NIH HHS · GM-34433 · United States
External Links
PubMed source DOI: 10.1083/jcb.105.2.669 PMC Full Text View journal details
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