Gene/Proteome Database (LMPD)

LMPD ID
LMP001729
Gene ID
4758
Species
Homo sapiens (Human)
Gene Name
sialidase 1 (lysosomal sialidase)
Gene Symbol
NEU1
Synonyms
NANH; NEU; SIAL1
Chromosome
6
Map Location
6p21.3
Summary
The protein encoded by this gene is a lysosomal enzyme that 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 is also referred to as 'protective protein'). Mutations in this gene can lead to sialidosis, a lysosomal storage disease that can be type 1 (cherry red spot-myoclonus syndrome or normosomatic type), which is late-onset, or type 2 (the dysmorphic type), which occurs at an earlier age with increased severity. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for NEU1

Proteins

sialidase-1 precursor
Refseq ID NP_000425
Protein GI 4557791
UniProt ID Q5JQI0
mRNA ID NM_000434
Length 415
RefSeq Status REVIEWED
MTGERPSTALPDRRWGPRILGFWGGCRVWVFAAIFLLLSLAASWSKAENDFGLVQPLVTMEQLLWVSGRQIGSVDTFRIPLITATPRGTLLAFAEARKMSSSDEGAKFIALRRSMDQGSTWSPTAFIVNDGDVPDGLNLGAVVSDVETGVVFLFYSLCAHKAGCQVASTMLVWSKDDGVSWSTPRNLSLDIGTEVFAPGPGSGIQKQREPRKGRLIVCGHGTLERDGVFCLLSDDHGASWRYGSGVSGIPYGQPKQENDFNPDECQPYELPDGSVVINARNQNNYHCHCRIVLRSYDACDTLRPRDVTFDPELVDPVVAAGAVVTSSGIVFFSNPAHPEFRVNLTLRWSFSNGTSWRKETVQLWPGPSGYSSLATLEGSMDGEEQAPQLYVLYEKGRNHYTESISVAKISVYGTL
sig_peptide: 1..47 calculated_mol_wt: 5249 peptide sequence: MTGERPSTALPDRRWGPRILGFWGGCRVWVFAAIFLLLSLAASWSKA mat_peptide: 48..415 product: sialidase-1 calculated_mol_wt: 40236 peptide sequence: ENDFGLVQPLVTMEQLLWVSGRQIGSVDTFRIPLITATPRGTLLAFAEARKMSSSDEGAKFIALRRSMDQGSTWSPTAFIVNDGDVPDGLNLGAVVSDVETGVVFLFYSLCAHKAGCQVASTMLVWSKDDGVSWSTPRNLSLDIGTEVFAPGPGSGIQKQREPRKGRLIVCGHGTLERDGVFCLLSDDHGASWRYGSGVSGIPYGQPKQENDFNPDECQPYELPDGSVVINARNQNNYHCHCRIVLRSYDACDTLRPRDVTFDPELVDPVVAAGAVVTSSGIVFFSNPAHPEFRVNLTLRWSFSNGTSWRKETVQLWPGPSGYSSLATLEGSMDGEEQAPQLYVLYEKGRNHYTESISVAKISVYGTL

Gene Information

Entrez Gene ID
4758
Gene Name
sialidase 1 (lysosomal sialidase)
Gene Symbol
NEU1
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0030054 IDA:HPA C cell junction
GO:0043231 IDA:HPA C intracellular membrane-bounded organelle
GO:0005764 IEA:Ensembl C lysosome
GO:0004308 IEA:Ensembl F exo-alpha-sialidase activity

KEGG Pathway Links

KEGG Pathway ID Description
hsa04142 Lysosome
hsa00600 Sphingolipid metabolism

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_116105 Glycosphingolipid metabolism
REACT_200874 Sialic acid metabolism

Domain Information

InterPro Annotations

Accession Description
IPR026856 Sialidase family
IPR011040 Sialidases

UniProt Annotations

Entry Information

Gene Name
sialidase 1 (lysosomal sialidase)
Protein Entry
NEUR1_HUMAN
UniProt ID
Q5JQI0
Species
Human

Identical and Related Proteins

Unique RefSeq proteins for LMP001729 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
4557791 RefSeq NP_000425 415 sialidase-1 precursor

Identical Sequences to LMP001729 proteins

Reference Database Accession Length Protein Name
GI:4557791 GenBank JAA04693.1 415 sialidase 1 (lysosomal sialidase) [Pan troglodytes]
GI:4557791 GenBank JAA11591.1 415 sialidase 1 (lysosomal sialidase) [Pan troglodytes]
GI:4557791 GenBank JAA28012.1 415 sialidase 1 (lysosomal sialidase) [Pan troglodytes]
GI:4557791 GenBank JAA33633.1 415 sialidase 1 (lysosomal sialidase) [Pan troglodytes]
GI:4557791 GenBank AIC49277.1 415 NEU1, partial [synthetic construct]
GI:4557791 RefSeq XP_004043753.1 415 PREDICTED: sialidase-1 [Gorilla gorilla gorilla]

Related Sequences to LMP001729 proteins

Reference Database Accession Length Protein Name
GI:4557791 EMBL CAG32998.1 415 NEU1 [Homo sapiens]
GI:4557791 GenBank AAP36936.1 416 Homo sapiens sialidase 1 (lysosomal sialidase), partial [synthetic construct]
GI:4557791 GenBank AAX43891.1 416 sialidase 1, partial [synthetic construct]
GI:4557791 GenBank AAX43892.1 416 sialidase 1, partial [synthetic construct]
GI:4557791 GenBank ACM85519.1 425 Sequence 11017 from patent US 6812339
GI:4557791 RefSeq XP_003831595.1 415 PREDICTED: sialidase-1 [Pan paniscus]