Xpnpep3 MGI Mouse Gene Detail - MGI:2445217 - X-prolyl aminopeptidase 3, mitochondrial

Symbol

Xpnpep3
Name

X-prolyl aminopeptidase 3, mitochondrial
Synonyms

E430012M05Rik

Feature Type

protein coding gene
IDs

MGI:2445217
NCBI Gene: 321003
Alliance

genome feature page
Transcription Start Sites

1 TSS

Sequence Map

Chr15:81284339-81341683 bp, + strand
From Ensembl annotation of GRCm39
View this region in JBrowse
Genome Browsers

Ensembl | UCSC | NCBI

Genetic Map

Chromosome 15, 38.08 cM
Mapping Data

1 experiment

SNPs within 2kb

1149 from dbSNP Build 142
Strain Annotations

19

For selected strains:

Strain	Gene Model ID	Feature Type	Coordinates
C57BL/6J	MGI_C57BL6J_2445217	protein coding gene	Chr15:81284282-81341683 (+)
129S1/SvImJ	ENSMUSG00200050073	protein coding gene	Chr15:78391156-78448502 (+)
A/J	ENSMUSG00195051378	protein coding gene	Chr15:78327948-78385294 (+)
AKR/J	ENSMUSG00220049265	protein coding gene	Chr15:78371127-78428476 (+)
BALB/cJ	ENSMUSG00180052774	protein coding gene	Chr15:78204932-78262279 (+)
C3H/HeJ	ENSMUSG00175041594	protein coding gene	Chr15:78515976-78573320 (+)
C57BL/6NJ	ENSMUSG00215049522	protein coding gene	Chr15:78301250-78358596 (+)
CAROLI/EiJ	MGP_CAROLIEiJ_G0020051	protein coding gene	Chr15:75418867-75473171 (+)
CAST/EiJ	ENSTCUG00005043406	protein coding gene	Chr15:77823297-77881134 (+)
CBA/J	ENSMUSG00210041523	protein coding gene	Chr15:78291067-78348410 (+)
DBA/2J	ENSMUSG00185049769	protein coding gene	Chr15:78314272-78371615 (+)
FVB/NJ	ENSMUSG00205025878	protein coding gene	Chr15:78034870-78092234 (+)
JF1/MsJ	ENSUMUG00000034794	protein coding gene	Chr15:77888278-77946521 (+)
LP/J	ENSMUSG00230051596	protein coding gene	Chr15:81638577-81695923 (+)
NOD/ShiLtJ	ENSMUSG00190049677	protein coding gene	Chr15:78308134-78365481 (+)
NZO/HlLtJ	ENSMUSG00225048684	protein coding gene	Chr15:81978500-82035862 (+)
PWK/PhJ	ENSLUMG00010040498	protein coding gene	Chr15:78109338-78167341 (+)
SPRET/EiJ	ENSMSPG00010048915	protein coding gene	Chr15:79754805-79812422 (+)
WSB/EiJ	ENSIUOG00005041331	protein coding gene	Chr15:78383493-78441146 (+)

Human Ortholog

XPNPEP3, X-prolyl aminopeptidase 3

Vertebrate Orthologs

3

Vertebrate Orthology Source

Alliance of Genome Resources

Human Ortholog

XPNPEP3, X-prolyl aminopeptidase 3
Synonyms

APP3, ICP55, NPHPL1
Links

HGNC:28052

NCBI Gene ID: 63929

UniProt: Q9NQH7
Chr Location

22q13.2; chr22:40857077-40932815 (+) GRCh38

MGI Vertebrate Homology

Xpnpep3 stringent orthology
1 human;1 mouse;1 rat;1 zebrafish
HCOP

vertebrate homology predictions: XPNPEP3
Gene Tree

Xpnpep3

Diseases

1 with human XPNPEP3 associations

				Human Disease	Mouse Models
				nephronophthisis-like nephropathy 1 IDs nephronophthisis-like nephropathy 1 DOID:0111117 OMIM:613159

Click on a disease name to see all genes associated with that disease.

References

1 with disease annotations

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Phenotype Summary

1 phenotype from 1 allele in 1 genetic background
32 phenotype references

Phenotype Overview

Blue squares indicate phenotypes directly attributed to mutations/alleles of this gene.

adipose tissue	behavior/neurological	cardiovascular system	cellular	craniofacial	digestive/alimentary system	embryo	endocrine/exocrine glands	growth/size/body	hearing/vestibular/ear	hematopoietic system	homeostasis/metabolism	integument	immune system	limbs/digits/tail	liver/biliary system	mortality/aging	muscle	nervous system	pigmentation	renal/urinary system	reproductive system	respiratory system	skeleton	taste/olfaction	neoplasm	vision/eye

Click cells to view annotations.

All Mutations and Alleles

35
Chemically induced (other)

1
Endonuclease-mediated

1
Gene trapped

29
Radiation induced

2
Targeted

2
Genomic Mutations

3 involving Xpnpep3
Incidental Mutations

Mutagenetix , APF
Find Mice (IMSR)

41 strains or lines available

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All GO Annotations

32
GO References

12

Molecular Function

carbohydrate derivative binding	cytoskeletal protein binding	DNA binding	enzyme regulator	hydrolase	ligase	lipid binding	oxidoreductase	RNA binding	signaling receptor activity	signaling receptor binding	transcription	transferase	transporter

Biological Process

carbohydrate derivative metabolism	cell differentiation	cell population proliferation	cellular component organization	DNA-templated transcription	establishment of localization	homeostatic process	immune system process	lipid metabolic process	programmed cell death	protein metabolic process	response to stimulus	signaling	system development

Cellular Component

cell projection	cytoplasmic vesicle	cytoskeleton	cytosol	endoplasmic reticulum	endosome	extracellular region	Golgi apparatus	mitochondrion	membraneless organelle	nucleus	organelle envelope	organelle lumen	plasma membrane	protein-containing complex	synapse	vacuole

Click cells to view annotations.

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Expression Overview

GXD's primary emphasis is on endogenous gene expression during development. Click on grid cells to view annotations.

Blue cells = expressed in wild-type.
Gray triangles = other expression annotations only
(e.g. absence of expression or data from mutants).

early conceptus	embryo ectoderm	embryo endoderm	embryo mesoderm	embryo mesenchyme	extraembryonic component	alimentary system	auditory system	branchial arches	cardiovascular system	connective tissue	endocrine system	exocrine system	hemolymphoid system	integumental system	limbs	liver and biliary system	musculoskeletal system	nervous system	olfactory system	reproductive system	respiratory system	urinary system	visual system

Click cells to view annotations.

Assay Results

651
Tissues

47
cDNA Data

11
Literature Summary

1

Images

Tissue x Stage Matrix

Other Mouse Links

Allen Institute

GEO

Expression Atlas
Other Vertebrate Links

Xenbase xpnpep3

ZFIN xpnpep3

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All Sequences

52
RefSeq

6
UniProt

2
CCDS

CCDS27668.1, CCDS84182.1

Ensembl

ENSMUSG00000022401 (Evidence)
NCBI Gene

321003

			Length	Strain/Species	Flank
genomic	ENSMUSG00000022401	Ensembl Gene Model \| MGI Sequence Detail	57345	C57BL/6J	± kb
transcript	ENSMUST00000163754	Ensembl \| MGI Sequence Detail	6058	Not Applicable
polypeptide	ENSMUSP00000132822	Ensembl \| MGI Sequence Detail	506	Not Applicable

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UniProt

2 Sequences
Protein Ontology

PR:000031424 Xaa-Pro aminopeptidase 3

(term hierarchy)
EC

3.4.11.9
InterPro Domains

IPR007865 Aminopeptidase P, N-terminal

IPR036005 Creatinase/aminopeptidase-like

IPR029149 Creatinase/Aminopeptidase P/Spt16, N-terminal

IPR000994 Peptidase M24

IPR052433 Xaa-Pro dipeptidase-like
GlyGen

B7ZMP1 1 site

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All nucleic 11

cDNA 11

Microarray probesets 3

Summaries

All 63

Developmental Gene Expression 1

Diseases 1

Gene Ontology 12

Phenotypes 32
Earliest

J:7688 Lane PW, et al., Association of megacolon with a new dominant spotting gene (Dom) in the mouse. J Hered. 1984 Nov-Dec;75(6):435-9
Latest

J:387680 Ahn B, et al., Disorganization of Transcriptional Regulation and Alteration of Keratin Family Gene Expression in Hairy Ear Mice. Genes (Basel). 2026 Jan 31;17(2)

TSS for Xpnpep3:

(View these features in JBrowse)

Transcription Start Site	Location	Distance from Gene 5'-end
Tssr132265	Chr15:81284333-81284416 (+)	36 bp

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources Funding Information Warranty Disclaimer, Privacy Notice, Licensing, & Copyright Send questions and comments to User Support.	last database update 09/08/2026 MGI 6.24