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Sialidase 1 (lysosomal sialidase)
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols NEU1 ; NANH; NEU; SIAL1
External IDs OMIM608272 MGI894696 HomoloGene375 GeneCards: NEU1 Gene
EC number
RNA expression pattern
PBB GE SERPING1 200986 at tn.png
More reference expression data
Species Human Mouse
Entrez 4758 18010
Ensembl ENSG00000184494 ENSMUSG00000007038
UniProt Q99519 O35657
RefSeq (mRNA) NM_000434 NM_010893
RefSeq (protein) NP_000425 NP_035023
Location (UCSC) Chr 6:
31.83 – 31.83 Mb
Chr 17:
34.93 – 34.94 Mb
PubMed search [1] [2]

Sialidase 1 (lysosomal sialidase), also known as NEU1 is a mammalian lysosomal neuraminidase enzyme which in humans is encoded by the NEU1 gene.[1][2]


The protein encoded by this gene encodes the lysosomal enzyme, which cleaves terminal sialic acid residues from substrates such as glycoproteins and glycolipids. In the lysosome, this enzyme is part of a heterotrimeric complex together with beta-galactosidase and cathepsin A (the latter also referred to as 'protective protein'). Mutations in this gene can lead to sialidosis.[1]

Clinical significance[edit]

Deficiencies in the human enzyme NEU1 leads to sialidosis, a rare lysosomal storage disease.[3] Sialidase has also been shown to enhance recovery from spinal cord contusion injury when injected in rats.[4]


NEU1 has been shown to interact with Cathepsin A.[5]


  1. ^ a b "Entrez Gene: NEU1 sialidase 1 (lysosomal sialidase)". 
  2. ^ Pshezhetsky AV, Richard C, Michaud L, Igdoura S, Wang S, Elsliger MA et al. (March 1997). "Cloning, expression and chromosomal mapping of human lysosomal sialidase and characterization of mutations in sialidosis". Nat. Genet. 15 (3): 316–20. doi:10.1038/ng0397-316. PMID 9054950. 
  3. ^ Seyrantepe V, Poupetova H, Froissart R, Zabot MT, Maire I, Pshezhetsky AV (November 2003). "Molecular pathology of NEU1 gene in sialidosis". Hum. Mutat. 22 (5): 343–52. doi:10.1002/humu.10268. PMID 14517945. 
  4. ^ Mountney A, Zahner MR, Lorenzini I, Oudega M, Schramm LP, Schnaar RL (June 2010). "Sialidase enhances recovery from spinal cord contusion injury". PNAS 22 (5): 343–52. doi:10.1073/pnas.1006683107. PMC 2895144. PMID 20534525. 
  5. ^ van der Spoel A, Bonten E, d'Azzo A (Mar 1998). "Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A". EMBO J. 17 (6): 1588–97. doi:10.1093/emboj/17.6.1588. PMC 1170506. PMID 9501080. 

Further reading[edit]