LONP2 - lon peptidase 2, peroxisomal Gene

Also Known as LONP; PLON; LONPL; PSLON

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 83752

About LONP2

Cytogenetic location: 16q12.1 Genomic coordinates (GRCh38): 16:48,244,300-48,363,003 (from NCBI)

This gene has 9 transcripts (splice variants), 213 orthologues and 1 paralogue. Ubiquitous expression in thyroid (RPKM 23.1), liver (RPKM 21.0) and 25 other tissues.

Summary

In human, peroxisomes function primarily to catalyze fatty acid beta-oxidation and, as a by-product, produce hydrogen peroxide and superoxide. The protein encoded by this gene is an ATP-dependent protease that likely plays a role in maintaining overall peroxisome homeostasis as well as proteolytically degrading peroxisomal proteins damaged by oxidation. The protein has an N-terminal Lon N substrate recognition domain, an ATPase domain, a proteolytic domain, and, in some isoforms, a C-terminal peroxisome targeting sequence. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Jan 2017]

LONP2 Products (3)

mRNA	Protein	Name
NM_001300948.3	NP_001287877.1	lon protease homolog 2, peroxisomal isoform 2
NM_001348078.2	NP_001335007.1	lon protease homolog 2, peroxisomal isoform 3
NM_031490.5	NP_113678.2	lon protease homolog 2, peroxisomal isoform 1

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables enzyme binding	IPI IPI: Inferred from physical interaction	18281296	GOA
enables peptidase activity	IDA IDA: Inferred from direct assay	18281296	GOA
enables protease binding	IPI IPI: Inferred from physical interaction	22002062	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	18281296	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in protein processing	IMP IMP: Inferred from mutant phenotype	18281296	GOA
involved in protein targeting to peroxisome	IMP IMP: Inferred from mutant phenotype	18281296	GOA
involved in regulation of fatty acid beta-oxidation	IMP IMP: Inferred from mutant phenotype	22002062	GOA

Cellular Component GO Annotation	Evidence	References	Source
located in peroxisome	IDA IDA: Inferred from direct assay	14561759	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

LONP2 Protein Structure

LON_substr_bdg

AAA

Lon_C

0
200
400
600
800
852 a.a.

Protein Preferred Names	Protein Names
lon protease homolog 2, peroxisomal	lon protease 2

LONP2 Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	LONP2	Q86WA8	SYCE1	Homo sapiens	Q8N0S2	Validated Y2H	32296183
Intra	LONP2	Q86WA8	SYCE1	Homo sapiens	Q8N0S2	Y2H Array	32296183
Intra	LONP2	Q86WA8	SYCE1	Homo sapiens	Q8N0S2	Y2H Prey Pooling	32296183

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases	Alias
Buratti-Harel Syndrome	BURHAS
Lateral Sinus Thrombosis	Thrombosis Of Lateral Venous Sinus Thrombosis Transverse Sinus
Codas Syndrome	Cerebral, Ocular, Dental, Auricular, And Skeletal Anomalies Syndrome Cerebral, Ocular, Dental, Auricular, And Skeletal Syndrome Cerebro-Oculo-Dento-Auriculo-Skeletal Syndrome Cerebrooculodentoauriculoskeletal Syndrome CODASS Cerebral, Ocular, Dental, Auricular, Skeletal Syndrome
Intracranial Sinus Thrombosis	Sinus Thrombosis, Intracranial
Peroxisomal Biogenesis Disorder	Zellweger Spectrum Disorders Peroxisome Biogenesis Disorder-Zellweger Syndrome Spectrum Disorders Of Peroxisome Biogenesis Zellweger Spectrum Zellweger Syndrome Spectrum Peroxisomal Biogenesis Disorders Pbd, Zss Pbd-Zsd Pbd-Zss Pbd-Zellweger Spectrum Disorder Peroxisomal Biogenesis Disorders, Zellweger Syndrome Spectrum Peroxisome Biogenesis Disorder Peroxisome Biogenesis Disorder Spectrum Peroxisome Biogenesis Disorders Zellweger Spectrum Disorder Hyperpipecolic Acidaemia
Peroxisome Biogenesis Disorder 1b	Peroxisome Biogenesis Disorder Infantile Refsum Disease Infantile Phytanic Acid Storage Disease PBD1B Refsum Disease, Infantile Adrenoleukodystrophy, Autosomal Neonatal Ird Mild Pbd-Zsd Mild Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder Pbd-Zsd Peroxisome Biogenesis Disorder Spectrum Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder Autosomal Neonatal Adrenoleukodystrophy Refsum Disease Infantile Peroxisome Biogenesis Disorders Peroxisome Biogenesis Disorder, Type 1b
Zellweger Syndrome	Cerebrohepatorenal Syndrome Zellweger Leukodystrophy Zs Congenital Iron Overload Chr Zws Severe Pbd-Zsd Severe Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS07735	LONP2 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Macaca mulatta	LONP2	VGNC	VGNC:74320
Bos taurus	LONP2	VGNC	VGNC:30948
Mus musculus	LONP2	MGD	MGI:1914137
Canis familiaris	LONP2	VGNC	VGNC:42733
Felis catus	LONP2	VGNC	VGNC:63255
Rattus norvegicus	LONP2	RGD	RGD:1305466