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Ngly1 Gene Detail
Summary
  • Symbol
    Ngly1
  • Name
    N-glycanase 1
  • Synonyms
    1110002C09Rik, Png1, PNGase
  • Feature Type
    protein coding gene
  • IDs
    MGI:1913276
    NCBI Gene: 59007
  • Alliance
    genome feature page
  • Transcription Start Sites
    2 TSS
Location &
Maps
more
  • Sequence Map
    Chr14:6157837-6220449 bp, - strand
    From NCBI annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
    Ensembl | UCSC | NCBI
  • Genetic Map
    Chromosome 14, 7.08 cM, cytoband A2
  • Mapping Data
    4 experiments
Strain
Comparison
more
  • SNPs within 2kb
    1296 from dbSNP Build 142
  • Strain Annotations
    19
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_1913276
 protein coding gene Chr14:6157837-6220483 (-)
129S1/SvImJ ENSMUSG00200023970
 protein coding gene Chr14:2720116-2782015 (-)
A/J ENSMUSG00195028805
 protein coding gene Chr14:3617079-3679731 (-)
AKR/J ENSMUSG00220023119
 protein coding gene Chr14:2767450-2830103 (-)
BALB/cJ ENSMUSG00180023429
 protein coding gene Chr14:2927167-2989815 (-)
C3H/HeJ ENSMUSG00175009285
 protein coding gene Chr14:2877691-2939595 (-)
C57BL/6NJ ENSMUSG00215015507
 protein coding gene Chr14:3194274-3256921 (-)
CAROLI/EiJ MGP_CAROLIEiJ_G0018968
 protein coding gene Chr14:9232481-9292979 (+)
CAST/EiJ ENSTCUG00005029848
 protein coding gene Chr14:2898937-2960580 (-)
CBA/J ENSMUSG00210032972
 protein coding gene Chr14:3099216-3161114 (-)
DBA/2J ENSMUSG00185035751
 protein coding gene Chr14:3104876-3166796 (-)
FVB/NJ ENSMUSG00205023420
 protein coding gene Chr14:3155054-3239936 (-)
JF1/MsJ ENSUMUG00000021051
 protein coding gene Chr14:3102692-3164614 (-)
LP/J ENSMUSG00230006752
 protein coding gene Chr14:4234204-4296122 (-)
NOD/ShiLtJ ENSMUSG00190031140
 protein coding gene Chr14:3189151-3251805 (-)
NZO/HlLtJ ENSMUSG00225036554
 protein coding gene Chr14:3601259-3663909 (-)
PWK/PhJ ENSLUMG00010031990
 protein coding gene Chr14:24447417-24509353 (-)
SPRET/EiJ ENSMSPG00010002366
 protein coding gene Chr14:3265903-3327976 (-)
WSB/EiJ ENSIUOG00005023500
 protein coding gene Chr14:2992536-3055174 (-)



Homology
more
  • Human Ortholog
    NGLY1, N-glycanase 1
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    NGLY1, N-glycanase 1
  • Synonyms
    CDDG, CDG1V, PNG-1, PNG1, PNGase
  • Links
    NCBI Gene ID: 55768
    UniProt: Q96IV0

  • Chr Location
    3p24.2; chr3:25718717-25790039 (-)  GRCh38
Human Diseases
more
  • Diseases
    1 with human NGLY1 associations

Human Disease Mouse Models
      
IDs
Click on a disease name to see all genes associated with that disease.

Mutations,
Alleles, and
Phenotypes
less
  • Phenotype Summary
    4 phenotypes from 4 alleles in 4 genetic backgrounds
    6 phenotypes from multigenic genotypes
    16 phenotype references
Phenotype Overview

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.
Mice homozygous for a knock-out allele exhibit dysregulation of the endoplasmic reticulum (ER)-associated degradation (ERAD) process.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
    25
  • GO References
    13
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.
Expression
less
Expression Overview

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
    765
  • Tissues
    16
  • cDNA Data
    60
  • Literature Summary
    2
  • Comparison Matrix
    Gene Expression + Phenotype
    • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic 59007 NCBI Gene Model | MGI Sequence Detail 62613 C57BL/6J ±  kb
    transcript NM_021504 RefSeq | MGI Sequence Detail 2901 ZRU/MplStud  
    polypeptide Q9JI78 UniProt | EBI | MGI Sequence Detail 651 Not Applicable  
    For the selected sequence
    Protein
    Information
    less
    Molecular
    Reagents
    less
    • All nucleic 62
      cDNA 61
      Primer pair 1

      Microarray probesets 3
    Other
    Accession IDs
    less
    MGI:1915786
    References
    more
    • Summaries
      All 63
      Developmental Gene Expression 2
      Gene Ontology 13
      Phenotypes 16
    • Earliest
      J:65050 Suzuki T, et al., PNG1, a yeast gene encoding a highly conserved peptide:N-glycanase. J Cell Biol. 2000 May 29;149(5):1039-52
    • Latest
      J:369943 Yang K, et al., The STING pathway drives noninflammatory neurodegeneration in NGLY1 deficiency. J Exp Med. 2025 Oct 6;222(10)
    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
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    Send questions and comments to User Support.     		last database update
    09/08/2026
    MGI 6.24     		The Jackson Laboratory