LONP2 - lon peptidase 2, peroxisomal Gene

Homo sapiens

Also known as LONP; PLON; LONPL; PSLON

Gene ID: 83752 | Gene type: protein coding

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	Reference	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	Reference	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	Reference	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

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

Bioactive Molecules (1)

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

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