LONP2 - lon peptidase 2, peroxisomal Gene

中文名称：lon 肽酶 2，过氧化物酶体

种属: Homo sapiens

同用名: LONP; PLON; LONPL; PSLON

基因 ID: 83752 | 基因类型: protein coding

关于 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.

功能概要

在人体中，过氧化物酶体的主要功能是催化脂肪酸 β-氧化，并作为副产物产生过氧化氢和超氧化物。该基因编码的蛋白质是一种 ATP 依赖性蛋白酶，可能在维持整体过氧化物酶体稳态以及蛋白水解降解因氧化受损的过氧化物酶体蛋白质方面发挥作用。该蛋白质具有一个 N 端 Lon N 底物识别结构域、一个 ATP 酶结构域、一个蛋白水解结构域，并且在某些亚型中，还具有一个 C 端过氧化物酶体靶向序列。可变剪接导致编码不同异构体的多个转录变体。[RefSeq 提供，2017 年 1 月]

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 基因产物(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

基因本体论

分子功能
生物过程
细胞组分

分子功能 GO 注释	逻辑证据	参考文献	来源
enables enzyme binding	IPI IPI: 通过物理相互作用推断	18281296	GOA
enables peptidase activity	IDA IDA: 通过直接分析推断	18281296	GOA
enables protease binding	IPI IPI: 通过物理相互作用推断	22002062	GOA
enables protein binding	IPI IPI: 通过物理相互作用推断	18281296	GOA

生物过程 GO 注释	逻辑证据	参考文献	来源
involved in protein processing	IMP IMP: 通过突变表型推断	18281296	GOA
involved in protein targeting to peroxisome	IMP IMP: 通过突变表型推断	18281296	GOA
involved in regulation of fatty acid beta-oxidation	IMP IMP: 通过突变表型推断	22002062	GOA

细胞组分 GO 注释	逻辑证据	参考文献	来源
located in peroxisome	IDA IDA: 通过直接分析推断	14561759	GOA

EXP：通过实验结果推断 IDA：通过直接分析推断 IPI：通过物理相互作用推断 IMP：通过突变表型推断 IGI：通过遗传相互作用推断 IEP：通过表达模式推断

LONP2 蛋白结构

LON_substr_bdg

AAA

Lon_C

0
200
400
600
800
852 a.a.

蛋白主名

其他名称

lon protease homolog 2, peroxisomal

lon protease 2

LONP2 蛋白互作信息

分类	蛋白名称	蛋白编号	互作蛋白	互作蛋白种属	互作蛋白编号	实验方法	参考文献
种属内	LONP2	Q86WA8	SYCE1	Homo sapiens	Q8N0S2	Validated Y2H	32296183
种属内	LONP2	Q86WA8	SYCE1	Homo sapiens	Q8N0S2	Y2H Array	32296183
种属内	LONP2	Q86WA8	SYCE1	Homo sapiens	Q8N0S2	Y2H Prey Pooling	32296183

种属间: 跨种属相互作用 种属内: 同种属相互作用

关联疾病

疾病名称

别名

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

疾病名称	别名
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

Cat. No.	产品名	作用方式	纯度	是否罕见病
HY-RS07735	LONP2 Human Pre-designed siRNA Set A	Pre-designed Set	/	否

直系同源

种属	基因名	来源	基因 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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