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Ngly1 N-glycanase 1 [ Mus musculus (house mouse) ]

Gene ID: 59007, updated on 11-Apr-2024

Summary

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Official Symbol
Ngly1provided by MGI
Official Full Name
N-glycanase 1provided by MGI
Primary source
MGI:MGI:1913276
See related
Ensembl:ENSMUSG00000021785 AllianceGenome:MGI:1913276
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
Png1; PNGase; 1110002C09Rik
Summary
Predicted to enable peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase activity. Involved in glycoprotein catabolic process. Located in cytoplasm. Is expressed in axial musculature; nervous system; thymus primordium; and vibrissa. Human ortholog(s) of this gene implicated in NGLY1-deficiency. Orthologous to human NGLY1 (N-glycanase 1). [provided by Alliance of Genome Resources, Apr 2022]
Expression
Ubiquitous expression in testis adult (RPKM 30.1), adrenal adult (RPKM 10.0) and 28 other tissues See more
Orthologs
human all
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Genomic context

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See Ngly1 in Genome Data Viewer
Location:
14 A1; 14 7.08 cM
Exon count:
12
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 14 NC_000080.7 (6157837..6220449, complement)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 14 NC_000080.6 (16249314..16311926)

Chromosome 14 - NC_000080.7Genomic Context describing neighboring genes Neighboring gene retinoic acid receptor, beta Neighboring gene STARR-seq mESC enhancer starr_36026 Neighboring gene predicted gene, 53820 Neighboring gene predicted gene, 46442 Neighboring gene STARR-seq mESC enhancer starr_36025 Neighboring gene STARR-positive B cell enhancer ABC_E1166 Neighboring gene STARR-seq mESC enhancer starr_36024 Neighboring gene topoisomerase (DNA) II beta Neighboring gene STARR-seq mESC enhancer starr_36023 Neighboring gene STARR-seq mESC enhancer starr_36022 Neighboring gene CapStarr-seq enhancer MGSCv37_chr14:17005903-17006137 Neighboring gene ribosomal protein L31, pseudogene 3 Neighboring gene 3-oxoacyl-ACP synthase, mitochondrial Neighboring gene ribosomal protein L4 pseudogene

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

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Related articles in PubMed

  1. JF1/B6F1 Ngly1(-/-) mouse as an isogenic animal model of NGLY1 deficiency. Asahina M, et al. Proc Jpn Acad Ser B Phys Biol Sci, 2021. PMID 33563880, Free PMC Article
  2. Alteration of N-glycosylation in the kidney in a mouse model of systemic lupus erythematosus: relative quantification of N-glycans using an isotope-tagging method. Hashii N, et al. Immunology, 2009 Mar. PMID 18710403, Free PMC Article
  3. Structural and biochemical studies of the C-terminal domain of mouse peptide-N-glycanase identify it as a mannose-binding module. Zhou X, et al. Proc Natl Acad Sci U S A, 2006 Nov 14. PMID 17088551, Free PMC Article
  4. Ngly1, a mouse gene encoding a deglycosylating enzyme implicated in proteasomal degradation: expression, genomic organization, and chromosomal mapping. Suzuki T, et al. Biochem Biophys Res Commun, 2003 May 2. PMID 12711318
  5. Purification and enzymatic properties of peptide:N-glycanase from C3H mouse-derived L-929 fibroblast cells. Possible widespread occurrence of post-translational remodification of proteins by N-deglycosylation. Suzuki T, et al. J Biol Chem, 1994 Jul 1. PMID 8021270

See all (41) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Comparative proteomics reveals elevated CCN2 in NGLY1-deficient cells.
    Title: Comparative proteomics reveals elevated CCN2 in NGLY1-deficient cells.
  2. Loss of peptide:N-glycanase causes proteasome dysfunction mediated by a sugar-recognizing ubiquitin ligase.
    Title: Loss of peptide:N-glycanase causes proteasome dysfunction mediated by a sugar-recognizing ubiquitin ligase.
  3. N-Glycanase 1 Transcriptionally Regulates Aquaporins Independent of Its Enzymatic Activity.
    Title: N-Glycanase 1 Transcriptionally Regulates Aquaporins Independent of Its Enzymatic Activity.
  4. findings reveal novel functions of the NGLY1-NRF1 (Nfe2l1) pathway in mitochondrial homeostasis and inflammation and uncover an unexpected therapeutic strategy using pharmacological activators of NRF2 for treating mitochondrial and immune dysregulation.
    Title: N-glycanase NGLY1 regulates mitochondrial homeostasis and inflammation through NRF1.
  5. These observations strongly suggest that the Ngly1- or Ngly1/Engase-deficient mice could serve as a valuable animal model for studies related to the pathogenesis of the NGLY1-deficiency, and that cytoplasmic ENGase represents one of the potential therapeutic targets for this genetic disorder.
    Title: Lethality of mice bearing a knockout of the Ngly1-gene is partially rescued by the additional deletion of the Engase gene.
  6. study underscores the functional importance of Ngly1 in the ERAD process and provides a potential mechanism underlying the phenotypic consequences of a newly emerging genetic disorder caused by mutation of the human NGLY1 gene
    Title: Endo-β-N-acetylglucosaminidase forms N-GlcNAc protein aggregates during ER-associated degradation in Ngly1-defective cells.
  7. cytoplasmic protein mouse p97(mp97) participates in the formation of a ternary complex containing mouse autocrine motility factor receptor (mAMFR), mp97, and peptide N-glycanase
    Title: The AAA ATPase p97 links peptide N-glycanase to the endoplasmic reticulum-associated E3 ligase autocrine motility factor receptor.
  8. enhances the activity of the mouse PNGase core domain, presumably by increasing the affinity of mouse PNGase for the glycan chains of misfolded glycoproteins
    Title: Structural and biochemical studies of the C-terminal domain of mouse peptide-N-glycanase identify it as a mannose-binding module.
  9. The crystal structure of the mouse peptide N-glycanase catalytic core in complex with the xeroderma pigmentosum group C binding domain from HR23B is described; the results suggest a co-evolution of ER-associated degradation and DNA repair pathways.
    Title: Structure of the mouse peptide N-glycanase-HR23 complex suggests co-evolution of the endoplasmic reticulum-associated degradation and DNA repair pathways.
  10. results suggested two possible pathways for the interaction between mPNGase and the proteasome; in one, mHR23B mediates the interaction between mPNGase and the proteasome; in an alternative pathway, mPNGase directly binds to the proteasome subunit, mS4
    Title: Multiple modes of interaction of the deglycosylation enzyme, mouse peptide N-glycanase, with the proteasome.
Submit: New GeneRIF Correction See all GeneRIFs (12)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables hydrolase activity IEA
Inferred from Electronic Annotation
more info
  
enables metal ion binding IEA
Inferred from Electronic Annotation
more info
  
enables peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase activity ISO
Inferred from Sequence Orthology
more info
  
enables peptide-N4-(N-acetyl-beta-glucosaminyl)asparagine amidase activity ISS
Inferred from Sequence or Structural Similarity
more info
 PubMed 
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
Process Evidence Code Pubs
involved_in glycoprotein catabolic process IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in glycoprotein catabolic process IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in glycoprotein catabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in protein deglycosylation IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in protein deglycosylation ISO
Inferred from Sequence Orthology
more info
  
Component Evidence Code Pubs
located_in collagen-containing extracellular matrix HDA PubMed 
located_in cytoplasm IDA
Inferred from Direct Assay
more info
 PubMed 
located_in cytoplasm ISO
Inferred from Sequence Orthology
more info
  
is_active_in cytosol IBA
Inferred from Biological aspect of Ancestor
more info
  
is_active_in nucleus IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in nucleus ISS
Inferred from Sequence or Structural Similarity
more info
 PubMed 

General protein information

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Preferred Names
peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase
Names
peptide N-glycanase homolog
NP_001349361.1
NP_001349362.1
NP_067479.2

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001362432.1 → NP_001349361.1  peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase isoform 2

    Status: VALIDATED

    Source sequence(s)
    AC173482, AK148931, BC028961
    Conserved Domains (4) summary
    pfam03835
    Location:316 → 400
    Rad4; Rad4 transglutaminase-like domain
    pfam01841
    Location:253 → 332
    Transglut_core; Transglutaminase-like superfamily
    pfam04721
    Location:435 → 629
    PAW; PNGase C-terminal domain, mannose-binding module PAW
    cl15262
    Location:26 → 90
    PUB; PNGase/UBA or UBX (PUB) domain of p97 adaptor proteins

  2. NM_001362433.1 → NP_001349362.1  peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase isoform 3

    Status: VALIDATED

    Source sequence(s)
    AC173482, AK028248, AK169205, BC028961
    Consensus CDS
    CCDS88581.1
    UniProtKB/TrEMBL
    A0A286YCI0
    Related
    ENSMUSP00000152998.2, ENSMUST00000224656.2
    Conserved Domains (4) summary
    cd10459
    Location:19 → 109
    PUB_PNGase; PNGase/UBA or UBX (PUB) domain of the P97 adaptor protein Peptide:N-glycanase (PNGase)
    pfam03835
    Location:335 → 420
    Rad4; Rad4 transglutaminase-like domain
    pfam01841
    Location:272 → 351
    Transglut_core; Transglutaminase-like superfamily
    pfam04721
    Location:413 → 593
    PAW; PNGase C-terminal domain, mannose-binding module PAW

  3. NM_021504.3 → NP_067479.2  peptide-N(4)-(N-acetyl-beta-glucosaminyl)asparagine amidase isoform 1

    See identical proteins and their annotated locations for NP_067479.2

    Status: VALIDATED

    Source sequence(s)
    AK028248
    Consensus CDS
    CCDS26832.1
    UniProtKB/Swiss-Prot
    Q8K113, Q9CTK3, Q9JI78
    Related
    ENSMUSP00000022310.7, ENSMUST00000022310.7
    Conserved Domains (5) summary
    cd10459
    Location:19 → 109
    PUB_PNGase; PNGase/UBA or UBX (PUB) domain of the P97 adaptor protein Peptide:N-glycanase (PNGase)
    COG1305
    Location:290 → 351
    YebA; Transglutaminase-like enzyme, putative cysteine protease [Posttranslational modification, protein turnover, chaperones]
    pfam01841
    Location:272 → 351
    Transglut_core; Transglutaminase-like superfamily
    pfam03835
    Location:335 → 419
    Rad4; Rad4 transglutaminase-like domain
    pfam04721
    Location:454 → 648
    PAW; PNGase C-terminal domain, mannose-binding module PAW

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000080.7 Reference GRCm39 C57BL/6J

    Range
    6157837..6220449 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q9JI78.2 GenPept UniProtKB/Swiss-Prot:Q9JI78

Gene LinkOut

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