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Sgsh Gene Detail
Summary
  • Symbol
    Sgsh
  • Name
    N-sulfoglucosamine sulfohydrolase (sulfamidase)
  • Synonyms
    4632406A19Rik, sulphamidase
  • Feature Type
    protein coding gene
  • IDs
    MGI:1350341
    NCBI Gene: 27029
  • Alliance
    gene page
  • Transcription Start Sites
    4 TSS
Location &
Maps
more
  • Sequence Map
    Chr11:119234315-119246336 bp, - strand
    From NCBI annotation of GRCm39
  • View this region in JBrowse
  • Genome Browsers
    Ensembl | UCSC | NCBI
  • Genetic Map
    Chromosome 11, 83.36 cM
  • Mapping Data
    3 experiments
Strain
Comparison
more
  • SNPs within 2kb
    917 from dbSNP Build 142
  • Strain Annotations
    18
  • PCR
    1
For selected strains:
Strain Gene Model ID Feature Type Coordinates Select Strains
C57BL/6J MGI_C57BL6J_1350341
 protein coding gene Chr11:119234251-119246362 (-)
129S1/SvImJ MGP_129S1SvImJ_G0019480
 protein coding gene Chr11:123726539-123738705 (-)
A/J MGP_AJ_G0019448
 protein coding gene Chr11:118243408-118255513 (-)
AKR/J MGP_AKRJ_G0019418
 protein coding gene Chr11:121802135-121814240 (-)
BALB/cJ MGP_BALBcJ_G0019423
 protein coding gene Chr11:118599742-118612844 (-)
C3H/HeJ MGP_C3HHeJ_G0019229
 protein coding gene Chr11:122117207-122134898 (-)
C57BL/6NJ MGP_C57BL6NJ_G0019872
 protein coding gene Chr11:127498403-127511009 (-)
CAROLI/EiJ MGP_CAROLIEiJ_G0017497
 protein coding gene Chr11:115238443-115250721 (-)
CAST/EiJ MGP_CASTEiJ_G0018783
 protein coding gene Chr11:123587299-123601250 (-)
CBA/J MGP_CBAJ_G0019200
 protein coding gene Chr11:132452154-132464383 (-)
DBA/2J MGP_DBA2J_G0019312
 protein coding gene Chr11:117613838-117626108 (-)
FVB/NJ MGP_FVBNJ_G0019304
 protein coding gene Chr11:117285995-117298467 (-)
LP/J MGP_LPJ_G0019384
 protein coding gene Chr11:124047924-124060156 (-)
NOD/ShiLtJ MGP_NODShiLtJ_G0019337
 protein coding gene Chr11:131383943-131397780 (-)
NZO/HlLtJ MGP_NZOHlLtJ_G0019911
 protein coding gene Chr11:122482958-122495057 (-)
PWK/PhJ MGP_PWKPhJ_G0018549
 protein coding gene Chr11:119022655-119034833 (-)
SPRET/EiJ MGP_SPRETEiJ_G0018341
 protein coding gene Chr11:122238666-122251999 (-)
WSB/EiJ MGP_WSBEiJ_G0018835
 protein coding gene Chr11:122465301-122479580 (-)



Homology
more
  • Human Ortholog
    SGSH, N-sulfoglucosamine sulfohydrolase
  • Vertebrate Orthologs
    3
Vertebrate Orthology Source
Alliance of Genome Resources
  • Human Ortholog
    SGSH, N-sulfoglucosamine sulfohydrolase
  • Synonyms
    HSS, MPS3A, SFMD
  • Links
    NCBI Gene ID: 6448
    UniProt: P51688

  • Chr Location
    17q25.3; chr17:80200673-80220923 (-)  GRCh38
Human Diseases
more
  • Diseases
    1 with Sgsh mouse models; 2 with human SGSH associations

Human Disease Mouse Models
      
IDs
 View 1 model
      
IDs
Click on a disease name to see all genes associated with that disease.

  • Mutations/Alleles
    1 with disease annotations
  • References
    1 with disease annotations
Mutations,
Alleles, and
Phenotypes
less
  • Phenotype Summary
    28 phenotypes from 2 alleles in 2 genetic backgrounds
    42 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 spontaneous allele die prematurely displaying low sulfamidase activity, variable lysosomal storage in various brain cell types and other tissues, heparan sulfate-uria, scruffy coats, corneal opacities, bladder distension, hydronephrosis, hepatosplenomegaly and bone deformities.
Gene Ontology
(GO)
Classifications
less
  • All GO Annotations
    19
  • GO References
    8
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
    802
  • Tissues
    96
  • cDNA Data
    29
  • Literature Summary
    4
  • Comparison Matrix
    Gene Expression + Phenotype
    • Sequences &
    Gene Models
    less
    Representative SequencesLengthStrain/SpeciesFlank
    genomic 27029 NCBI Gene Model | MGI Sequence Detail 12022 C57BL/6J ±  kb
    transcript NM_018822 RefSeq | MGI Sequence Detail 4324 ZRU/MplStud  
    polypeptide NP_061292 RefSeq | MGI Sequence Detail 502 ZRU/MplStud  
    For the selected sequence
    Protein
    Information
    less
    • UniProt
      8 Sequences
    • EC
      3.10.1.1
    • InterPro Domains
      IPR017850 Alkaline-phosphatase-like, core domain superfamily
      IPR032506 N-sulphoglucosamine sulphohydrolase, C-terminal
      IPR024607 Sulfatase, conserved site
      IPR000917 Sulfatase, N-terminal
    • GlyGen
      Q9EQ08 2 sites, 2 N-linked glycans (2 sites)
    Molecular
    Reagents
    less
    • All nucleic 31
      cDNA 29
      Primer pair 1
      Other 1

      Microarray probesets 4
    Other
    Accession IDs
    less
    MGI:2443648
    References
    more
    • Summaries
      All 72
      Developmental Gene Expression 4
      Diseases 1
      Gene Ontology 8
      Phenotypes 42
    • Earliest
      J:72142 Bhaumik M, et al., A mouse model for mucopolysaccharidosis type III A (Sanfilippo syndrome). Glycobiology. 1999 Dec;9(12):1389-96
    • Latest
      J:345621 Adams DJ, et al., Genetic determinants of micronucleus formation in vivo. Nature. 2024 Mar;627(8002):130-136
    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)
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    Funding Information
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    03/25/2025
    MGI 6.24     		The Jackson Laboratory