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lon peptidase 1, mitochondrial

Summary

Gene Symbol

LONP1

Organism

Homo sapiens (human)

External Links

NCBI Gene

9361

PubChem

9361

Alliance of Genome Resources

HGNC:9479

JoGo

LONP1

TogoVar

LONP1

Annotation

Keyword

3D-structure
ATP-binding
Alternative splicing
Cataract
DNA-binding
Deafness
Disease variant
Dwarfism
Mitochondrion
Proteomics identification
Reference proteome
Serine protease
Transit peptide

Proteins

Displaying 1 entry
UniProt	Protein Name
P36776	LONHs Lon protease-like protein Mitochondrial ATP-dependent protease Lon Serine protease 15

Annotation information from UniProt.

Gene Ontology (GO)

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GO Term	Evidence Code	PMID
mitochondrial genome maintenance	NAS	17420247
response to hypoxia	IEP	17418790
protein quality control for misfolded or incompletely synthesized proteins	IBA
mitochondrion organization	IMP	15683722
mitochondrion organization	IBA

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

organonitrogen compound metabolic process [inference]

protein metabolic process [inference]

proteolysis [inference]

proteolysis involved in protein catabolic process

protein quality control for misfolded or incompletely synthesized proteins

modification-dependent protein catabolic process [inference]

oxidation-dependent protein catabolic process

protein catabolic process

mitochondrial protein catabolic process

organonitrogen compound catabolic process [inference]

protein catabolic process

mitochondrial protein catabolic process

catabolic process [inference]

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process

mitochondrial protein catabolic process

modification-dependent macromolecule catabolic process [inference]

modification-dependent protein catabolic process [inference]

oxidation-dependent protein catabolic process

organonitrogen compound catabolic process [inference]

protein catabolic process

mitochondrial protein catabolic process

cellular metabolic process [inference]

cellular aromatic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

heterocycle metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

primary metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

protein metabolic process [inference]

proteolysis [inference]

proteolysis involved in protein catabolic process

protein quality control for misfolded or incompletely synthesized proteins

modification-dependent protein catabolic process [inference]

oxidation-dependent protein catabolic process

protein catabolic process

mitochondrial protein catabolic process

organic substance metabolic process [inference]

macromolecule metabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process

mitochondrial protein catabolic process

modification-dependent macromolecule catabolic process [inference]

modification-dependent protein catabolic process [inference]

oxidation-dependent protein catabolic process

protein metabolic process [inference]

proteolysis [inference]

proteolysis involved in protein catabolic process

protein quality control for misfolded or incompletely synthesized proteins

modification-dependent protein catabolic process [inference]

oxidation-dependent protein catabolic process

protein catabolic process

mitochondrial protein catabolic process

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

organic cyclic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

organonitrogen compound metabolic process [inference]

protein metabolic process [inference]

proteolysis [inference]

proteolysis involved in protein catabolic process

protein quality control for misfolded or incompletely synthesized proteins

modification-dependent protein catabolic process [inference]

oxidation-dependent protein catabolic process

protein catabolic process

mitochondrial protein catabolic process

organonitrogen compound catabolic process [inference]

protein catabolic process

mitochondrial protein catabolic process

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process

mitochondrial protein catabolic process

modification-dependent macromolecule catabolic process [inference]

modification-dependent protein catabolic process [inference]

oxidation-dependent protein catabolic process

organonitrogen compound catabolic process [inference]

protein catabolic process

mitochondrial protein catabolic process

cellular process [inference]

cellular metabolic process [inference]

cellular aromatic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

heterocycle metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

DNA metabolic process [inference]

mitochondrial DNA metabolic process

cellular response to stimulus [inference]

cellular response to stress [inference]

cellular response to chemical stress [inference]

cellular response to oxidative stress

cellular response to chemical stimulus [inference]

cellular response to chemical stress [inference]

cellular response to oxidative stress

cellular component organization or biogenesis [inference]

cellular component organization [inference]

organelle organization [inference]

mitochondrion organization

mitochondrial genome maintenance

cellular component assembly [inference]

protein-containing complex assembly [inference]

chaperone-mediated protein complex assembly

protein-containing complex organization [inference]

protein-containing complex assembly [inference]

chaperone-mediated protein complex assembly

response to stimulus [inference]

response to stress [inference]

response to hypoxia

response to oxidative stress [inference]

cellular response to oxidative stress

cellular response to stress [inference]

cellular response to chemical stress [inference]

cellular response to oxidative stress

response to abiotic stimulus [inference]

response to oxygen levels [inference]

response to decreased oxygen levels [inference]

response to hypoxia

response to endogenous stimulus [inference]

response to hormone

response to chemical [inference]

response to organic substance [inference]

response to hormone

response to inorganic substance [inference]

response to metal ion [inference]

response to aluminum ion

cellular response to chemical stimulus [inference]

cellular response to chemical stress [inference]

cellular response to oxidative stress

cellular response to stimulus [inference]

cellular response to stress [inference]

cellular response to chemical stress [inference]

cellular response to oxidative stress

cellular response to chemical stimulus [inference]

cellular response to chemical stress [inference]

cellular response to oxidative stress

biological regulation [inference]

regulation of biological process [inference]

regulation of signaling [inference]

regulation of signal transduction [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

regulation of insulin receptor signaling pathway [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of signaling [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of biological process [inference]

negative regulation of signaling [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of cellular process [inference]

negative regulation of cell communication [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of cellular response to insulin stimulus [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of response to stimulus [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of cellular response to insulin stimulus [inference]

negative regulation of insulin receptor signaling pathway

regulation of response to stimulus [inference]

regulation of signal transduction [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

regulation of insulin receptor signaling pathway [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of response to stimulus [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of cellular response to insulin stimulus [inference]

negative regulation of insulin receptor signaling pathway

regulation of cellular response to insulin stimulus [inference]

negative regulation of cellular response to insulin stimulus [inference]

negative regulation of insulin receptor signaling pathway

regulation of cellular process [inference]

regulation of cell communication [inference]

regulation of signal transduction [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

regulation of insulin receptor signaling pathway [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of cell communication [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of cellular process [inference]

negative regulation of cell communication [inference]

negative regulation of signal transduction [inference]

negative regulation of insulin receptor signaling pathway

negative regulation of cellular response to insulin stimulus [inference]

negative regulation of insulin receptor signaling pathway

regulation of cellular response to insulin stimulus [inference]

negative regulation of cellular response to insulin stimulus [inference]

negative regulation of insulin receptor signaling pathway

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GO Term	Evidence Code	PMID
nucleoplasm	IDA
mitochondrion	IDA	8248235
mitochondrion	HTP	34800366
mitochondrial matrix	TAS
mitochondrial matrix	IMP	12198491

GO Hierarchy

cellular component

cellular anatomical entity [inference]

nucleoid [inference]

mitochondrial nucleoid

membrane

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

non-membrane-bounded organelle [inference]

intracellular non-membrane-bounded organelle [inference]

mitochondrial nucleoid

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular non-membrane-bounded organelle [inference]

mitochondrial nucleoid

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GO Term	Evidence Code	PMID
mitochondrial promoter sequence-specific DNA binding	IDA	9485316
single-stranded DNA binding	IBA
single-stranded RNA binding	IDA	14739292
ATP-dependent peptidase activity	TAS
ATP-dependent peptidase activity	IDA	17420247 37327776 8248235

GO Hierarchy

molecular function

catalytic activity [inference]

hydrolase activity [inference]

peptidase activity [inference]

endopeptidase activity [inference]

serine-type endopeptidase activity

ATP-dependent peptidase activity

serine-type peptidase activity [inference]

serine-type endopeptidase activity

hydrolase activity, acting on acid anhydrides [inference]

hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides [inference]

pyrophosphatase activity [inference]

ribonucleoside triphosphate phosphatase activity [inference]

ATP hydrolysis activity

serine hydrolase activity [inference]

serine-type peptidase activity [inference]

serine-type endopeptidase activity

catalytic activity, acting on a protein [inference]

peptidase activity [inference]

endopeptidase activity [inference]

serine-type endopeptidase activity

ATP-dependent peptidase activity

serine-type peptidase activity [inference]

serine-type endopeptidase activity

binding [inference]

protein binding

enzyme binding [inference]

DNA polymerase binding

protein domain specific binding [inference]

PH domain binding

small molecule binding [inference]

heterocyclic compound binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ion binding [inference]

anion binding [inference]

purine ribonucleoside triphosphate binding [inference]

ATP binding

ADP binding

organic cyclic compound binding [inference]

nucleic acid binding [inference]

transcription regulatory region nucleic acid binding [inference]

transcription cis-regulatory region binding [inference]

mitochondrial promoter sequence-specific DNA binding

DNA binding [inference]

double-stranded DNA binding [inference]

sequence-specific double-stranded DNA binding [inference]

transcription cis-regulatory region binding [inference]

mitochondrial promoter sequence-specific DNA binding

single-stranded DNA binding

sequence-specific DNA binding

sequence-specific double-stranded DNA binding [inference]

transcription cis-regulatory region binding [inference]

mitochondrial promoter sequence-specific DNA binding

G-quadruplex DNA binding

RNA binding [inference]

single-stranded RNA binding

nucleoside phosphate binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

purine ribonucleoside triphosphate binding [inference]

ATP binding

carbohydrate derivative binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP-dependent activity [inference]

ATP-dependent peptidase activity

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

LON protease

Functional Category

K: Transcription
O: Posttranslational modification, protein turnover, chaperones
T: Signal transduction mechanisms

Displaying **all 7** entries
Gene Ontology
ATP binding
hydrolase activity
nucleotide binding
protein quality control for misfolded or incompletely synthesized proteins
proteolysis
serine-type endopeptidase activity
serine-type peptidase activity

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InterPro
AAA+ ATPase domain
ATPase, AAA-type, core
Lon protease, N-terminal domain superfamily
Lon protease, N-terminal domain
Lon protease, bacterial/eukaryotic-type
Lon protease
P-loop containing nucleoside triphosphate hydrolase
PUA-like superfamily
Peptidase S16, Lon proteolytic domain
Peptidase S16, active site

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0111274	CODAS syndrome	Alliance of Genome Resources

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

LM-GlycoRepo

All Resources

Other Information

Tools

Search Tools

Guidelines

Summary

External Links

Annotation

OrthoDB (Group)

International Collaboration

Acknowledgements