##gff-version 3
O75439	UniProtKB	Transit peptide	1	43	.	.	.	Note=Mitochondrion;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:19892738;Dbxref=PMID:19892738	
O75439	UniProtKB	Chain	44	489	.	.	.	ID=PRO_0000026777;Note=Mitochondrial-processing peptidase subunit beta	
O75439	UniProtKB	Active site	104	104	.	.	.	Note=Proton acceptor;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU10096	
O75439	UniProtKB	Binding site	101	101	.	.	.	Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU10096	
O75439	UniProtKB	Binding site	105	105	.	.	.	Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU10096	
O75439	UniProtKB	Binding site	181	181	.	.	.	Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU10096	
O75439	UniProtKB	Site	191	191	.	.	.	Note=Required for the specific determination of the substrate cleavage site;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q03346	
O75439	UniProtKB	Site	195	195	.	.	.	Note=Required for the specific determination of the substrate cleavage site;Ontology_term=ECO:0000250;evidence=ECO:0000250|UniProtKB:Q03346	
O75439	UniProtKB	Natural variant	175	175	.	.	.	ID=VAR_080804;Note=In MMDS6%3B exhibits temperature-sensitive defect in presequence processing activity%2C when tested in yeast. R->C;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:29576218;Dbxref=dbSNP:rs145596167,PMID:29576218	
O75439	UniProtKB	Natural variant	175	175	.	.	.	ID=VAR_080805;Note=In MMDS6%3B exhibits temperature-sensitive defect in presequence processing activity%2C when tested in yeast. R->H;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:29576218;Dbxref=dbSNP:rs200188353,PMID:29576218	
O75439	UniProtKB	Natural variant	177	177	.	.	.	ID=VAR_080806;Note=In MMDS6%3B exhibits temperature-sensitive defect in presequence processing activity%2C when tested in yeast. V->G;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:29576218;Dbxref=dbSNP:rs1436866272,PMID:29576218	
O75439	UniProtKB	Natural variant	201	201	.	.	.	ID=VAR_080807;Note=In MMDS6%3B exhibits temperature-sensitive defect in presequence processing activity%2C when tested in yeast. A->P;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:29576218;Dbxref=dbSNP:rs146343535,PMID:29576218	
O75439	UniProtKB	Natural variant	396	396	.	.	.	ID=VAR_051572;Note=E->D;Dbxref=dbSNP:rs3087615	
O75439	UniProtKB	Natural variant	422	422	.	.	.	ID=VAR_080808;Note=In MMDS6%3B small decrease in protein level%3B impaired frataxin/FXN processing%2C leading to the accumulation of an intermediate form%2C called iFXN. I->T;Ontology_term=ECO:0000269;evidence=ECO:0000269|PubMed:29576218;Dbxref=dbSNP:rs1461200360,PMID:29576218	
O75439	UniProtKB	Sequence conflict	16	17	.	.	.	Note=RR->GG;Ontology_term=ECO:0000305;evidence=ECO:0000305