Lon Lon protease [Drosophila melanogaster (fruit fly)] - Gene

Summary

Official Symbol: Lonprovided by FlyBase
Official Full Name: Lon proteaseprovided by FlyBase
Primary source: FLYBASE:FBgn0036892
Locus tag: Dmel_CG8798
See related: AllianceGenome:FB:FBgn0036892
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Drosophila melanogaster
Lineage: Eukaryota; Metazoa; Ecdysozoa; Arthropoda; Hexapoda; Insecta; Pterygota; Neoptera; Endopterygota; Diptera; Brachycera; Muscomorpha; Ephydroidea; Drosophilidae; Drosophila; Sophophora
Also known as: anon-WO0118547.350; CG8798; dLon P1; Dmel\CG8798; LON; LONP; LONP1
Summary: Enables serine hydrolase activity. Involved in mitochondrion organization and positive regulation of protein catabolic process. Located in mitochondrion. Is expressed in several structures, including Malpighian tubule primordium; adult head; embryonic/larval midgut primordium; gut section; and mesoderm. Used to study CODAS syndrome. Human ortholog(s) of this gene implicated in CODAS syndrome. Orthologous to human LONP1 (lon peptidase 1, mitochondrial). [provided by Alliance of Genome Resources, Nov 2024]
Orthologs: all
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Annotation release	Status	Assembly	Chr	Location
Release 6.54	current	Release 6 plus ISO1 MT (GCF_000001215.4)	3L	NT_037436.4 (19607857..19612199, complement)
Release 5.57	previous assembly	Release 5 (GCF_000001215.2)	3L	NT_037436.3 (19600957..19605299, complement)

Chromosome 3L - NT_037436.4 Genomic Context describing neighboring genes

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition.
Title: Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition.
Lon regulates mitochondrial transcription by stabilizing the mitochondrial TFAMmtDNA ratio via selective degradation of TFAM.
Title: Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM).

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

Function	Evidence Code	Pubs
enables ATP binding	IEA Inferred from Electronic Annotation more info
enables ATP hydrolysis activity	IEA Inferred from Electronic Annotation more info
enables ATP-dependent peptidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables ATP-dependent peptidase activity	IEA Inferred from Electronic Annotation more info
enables serine hydrolase activity	HDA more info	PubMed
enables serine-type endopeptidase activity	IEA Inferred from Electronic Annotation more info
enables single-stranded DNA binding	IBA Inferred from Biological aspect of Ancestor more info

Process	Evidence Code	Pubs
involved_in chaperone-mediated protein complex assembly	IBA Inferred from Biological aspect of Ancestor more info
involved_in mitochondrion organization	IBA Inferred from Biological aspect of Ancestor more info
involved_in mitochondrion organization	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in positive regulation of protein catabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in protein catabolic process	IEA Inferred from Electronic Annotation more info
involved_in protein quality control for misfolded or incompletely synthesized proteins	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein quality control for misfolded or incompletely synthesized proteins	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
is_active_in mitochondrial matrix	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in peroxisome	ISS Inferred from Sequence or Structural Similarity more info	PubMed

NP_649133.1

NP_730435.2

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The following sections contain reference sequences that belong to a specific genome build. Explain