Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase
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Stadler, SC, Polanetz, R, Meier, S, Mayerhofer, PU, Herrmann, JM, Anslinger, K, Roscher, AA, Roschinger, W and Holzinger, A (2005) Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase Biochem Biophys Res Commun, 334. pp. 939-946.
Full text not available from this repository.Abstract
Inherited deficiency of 3-methylcrotonyl-CoA carboxylase (MCC), an enzyme of leucine degradation, is an organic acidemia detectable by expanded newborn screening with a variable phenotype that ranges from asymptomatic to death in infancy. Here, we show that the two subunits of the enzyme (MCCalpha; MCCbeta) are imported into the mitochondrial matrix by the classical pathway involving cleavable amino-terminal targeting presequences. We identified the cleavage sites (Tyr41/Thr42 and Ala22/Tyr23 for MCCalpha and MCCbeta, respectively) of the targeting signals and the amino-termini of the mature polypeptides of MCC and propionyl-CoA carboxylase, a mitochondrial paralog. The amino-termini containing 39 (MCCalpha) or 20 amino acids (MCCbeta) were both necessary and sufficient for targeting. Structural requirements for mitochondrial import were defined by site-directed mutagenesis. Our studies provide the prerequisite to understand the impact of specific mutations on the clinical phenotype of MCC deficiency.
| Item Type: | Article | ||||||||||||||||||||||||||||||
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| Date : | 2005 | ||||||||||||||||||||||||||||||
| DOI : | 10.1016/j.bbrc.2005.06.190 | ||||||||||||||||||||||||||||||
| Uncontrolled Keywords : | Amino Acid Sequence Carbon-Carbon Ligases/chemistry/deficiency/genetics/*metabolism Carrier Proteins/metabolism Humans Kidney/chemistry Mitochondria/*metabolism Molecular Sequence Data Protein Transport/*physiology Recombinant Fusion Proteins/metabolism Saccharomyces cerevisiae/metabolism | ||||||||||||||||||||||||||||||
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| Depositing User : | Symplectic Elements | ||||||||||||||||||||||||||||||
| Date Deposited : | 17 May 2017 10:42 | ||||||||||||||||||||||||||||||
| Last Modified : | 16 Jan 2019 18:04 | ||||||||||||||||||||||||||||||
| URI: | http://epubs.surrey.ac.uk/id/eprint/828853 |
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