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Serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols SERPING1 ; C1IN; C1INH; C1NH; HAE1; HAE2
External IDs OMIM606860 MGI894696 HomoloGene44 GeneCards: SERPING1 Gene
RNA expression pattern
PBB GE SERPING1 200986 at tn.png
More reference expression data
Species Human Mouse
Entrez 710 12258
Ensembl ENSG00000149131 ENSMUSG00000023224
UniProt P05155 P97290
RefSeq (mRNA) NM_000062 NM_009776
RefSeq (protein) NP_000053 NP_033906
Location (UCSC) Chr 11:
57.36 – 57.38 Mb
Chr 2:
84.77 – 84.78 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, Qu J, Leclerc D, Gravel R, Dallaire L, Potier M (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. (ENGLAND) 17 (6): 1588–97. doi:10.1093/emboj/17.6.1588. ISSN 0261-4189. PMC 1170506. PMID 9501080. 

Further reading[edit]