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Q3UDW8

- HGNAT_MOUSE

UniProt

Q3UDW8 - HGNAT_MOUSE

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Protein

Heparan-alpha-glucosaminide N-acetyltransferase

Gene
Hgsnat, D8Ertd354e, Tmem76
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

Functioni

Lysosomal acetyltransferase that acetylates the non-reducing terminal alpha-glucosamine residue of intralysosomal heparin or heparan sulfate, converting it into a substrate for luminal alpha-N-acetyl glucosaminidase.1 Publication

Catalytic activityi

Acetyl-CoA + heparan sulfate alpha-D-glucosaminide = CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide.1 Publication

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Active sitei290 – 2901 By similarity

GO - Molecular functioni

  1. heparan-alpha-glucosaminide N-acetyltransferase activity Source: UniProtKB-EC
  2. transferase activity, transferring acyl groups Source: UniProtKB

GO - Biological processi

  1. lysosomal transport Source: UniProtKB
  2. metabolic process Source: GOC
  3. protein oligomerization Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Acyltransferase, Transferase

Enzyme and pathway databases

ReactomeiREACT_198967. HS-GAG degradation.

Names & Taxonomyi

Protein namesi
Recommended name:
Heparan-alpha-glucosaminide N-acetyltransferase (EC:2.3.1.78)
Alternative name(s):
Transmembrane protein 76
Gene namesi
Name:Hgsnat
Synonyms:D8Ertd354e, Tmem76
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 8

Organism-specific databases

MGIiMGI:1196297. Hgsnat.

Subcellular locationi

Lysosome membrane; Multi-pass membrane protein
Note: Colocalizes with the lysosomal marker LAMP2. The signal peptide is not cleaved upon translocation into the endoplasmic reticulum; the precursor is probably targeted to the lysosomes via the adapter protein complex-mediated pathway that involves tyrosine- and/or dileucine-based conserved amino acid motifs in the last C-terminus 16-amino acid domain By similarity.1 Publication

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 185185Lumenal, vesicle Reviewed predictionAdd
BLAST
Transmembranei186 – 20621Helical; Reviewed predictionAdd
BLAST
Topological domaini207 – 26862Cytoplasmic Reviewed predictionAdd
BLAST
Transmembranei269 – 28921Helical; Reviewed predictionAdd
BLAST
Topological domaini290 – 2956Lumenal, vesicle Reviewed prediction
Transmembranei296 – 31621Helical; Reviewed predictionAdd
BLAST
Topological domaini317 – 33822Cytoplasmic Reviewed predictionAdd
BLAST
Transmembranei339 – 35921Helical; Reviewed predictionAdd
BLAST
Topological domaini360 – 3678Lumenal, vesicle Reviewed prediction
Transmembranei368 – 38821Helical; Reviewed predictionAdd
BLAST
Topological domaini389 – 41325Cytoplasmic Reviewed predictionAdd
BLAST
Transmembranei414 – 43421Helical; Reviewed predictionAdd
BLAST
Topological domaini435 – 49359Lumenal, vesicle Reviewed predictionAdd
BLAST
Transmembranei494 – 51421Helical; Reviewed predictionAdd
BLAST
Topological domaini515 – 5228Cytoplasmic Reviewed prediction
Transmembranei523 – 54321Helical; Reviewed predictionAdd
BLAST
Topological domaini544 – 55714Lumenal, vesicle Reviewed predictionAdd
BLAST
Transmembranei558 – 57821Helical; Reviewed predictionAdd
BLAST
Topological domaini579 – 5857Cytoplasmic Reviewed prediction
Transmembranei586 – 60621Helical; Reviewed predictionAdd
BLAST
Topological domaini607 – 62721Lumenal, vesicle Reviewed predictionAdd
BLAST
Transmembranei628 – 64821Helical; Reviewed predictionAdd
BLAST
Topological domaini649 – 6568Cytoplasmic Reviewed prediction

GO - Cellular componenti

  1. integral component of membrane Source: UniProtKB-KW
  2. lysosomal membrane Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Lysosome, Membrane

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 656656Heparan-alpha-glucosaminide N-acetyltransferasePRO_0000273154Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi137 – 1371N-linked (GlcNAc...) Reviewed prediction
Disulfide bondi146 ↔ 455 By similarity
Glycosylationi157 – 1571N-linked (GlcNAc...)1 Publication
Modified residuei240 – 2401Phosphoserine By similarity
Modified residuei249 – 2491Phosphotyrosine1 Publication

Post-translational modificationi

Undergoes intralysosomal proteolytic cleavage; occurs within the end of the first and/or the beginning of the second luminal domain and is essential for the activation of the enzyme By similarity.
Glycosylated By similarity.

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein

Proteomic databases

PaxDbiQ3UDW8.
PRIDEiQ3UDW8.

PTM databases

PhosphoSiteiQ3UDW8.

Expressioni

Gene expression databases

BgeeiQ3UDW8.
CleanExiMM_HGSNAT.
GenevestigatoriQ3UDW8.

Interactioni

Subunit structurei

Homooligomer. Homooligomerization is necessary for enzyme activity By similarity.

Structurei

3D structure databases

ProteinModelPortaliQ3UDW8.

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni641 – 65616Lysosomal targeting region By similarityAdd
BLAST

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiCOG4299.
GeneTreeiENSGT00390000001491.
HOGENOMiHOG000006803.
HOVERGENiHBG081599.
InParanoidiQ3UDW8.
KOiK10532.
OMAiNSERCYH.
OrthoDBiEOG7PS1F9.
TreeFamiTF324790.

Family and domain databases

InterProiIPR012429. DUF1624.
[Graphical view]
PfamiPF07786. DUF1624. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative initiation. Align

Isoform 1 (identifier: Q3UDW8-1) [UniParc]FASTAAdd to Basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

MTGGSSSRRR RAEERSSAAG TERNSRREAV GGMGAGPALA ALLLAGSVLS    50
ATLLAPGRRA EPDLDEKRNV ELKMDQALLL IHNELLGTSL TVYWKSDDCY 100
QCTFQPLANV SHGGKPAKPS VAPVSVSTQH GSILQVNSTS EERAACRLEY 150
KFGEFGNYSL LVQHASSGAN KIACDIIVNE NPVDSNLPVS IAFLVGLALI 200
VAVSLLRLLL SLDDVNNWIS KTIASRETDR LINSELGSPS RADPLSADYQ 250
PETRRSSANR LRCVDTFRGL ALVLMVFVNY GGGKYWYFKH SSWNGLTVAD 300
LVFPWFVFIM GTSIFLSMTS ILQRGCSKLK LLGKIVWRSF LLICIGVIIV 350
NPNYCLGPLS WDKVRIPGVL QRLGVTYFVV AVLEFFFWKP VPDSCTLESS 400
CFSLRDITSS WPQWLTILTL ESIWLALTFF LPVPGCPTGY LGPGGIGDLG 450
KYPHCTGGAA GYIDRLLLGD NHLYQHPSST VLYHTEVAYD PEGVLGTINS 500
IVMAFLGVQA GKILVYYKDQ TKAILTRFAA WCCILGLISI VLTKVSANEG 550
FIPINKNLWS ISYVTTLSCF AFFILLILYP VVDVKGLWTG TPFFYPGMNS 600
ILVYVGHEVL ENYFPFQWKL ADEQSHKEHL IQNIVATALW VLIAYVLYKK 650
KLFWKI 656
Length:656
Mass (Da):72,504
Last modified:July 27, 2011 - v2
Checksum:i8FAE5EBECED5CCE3
GO
Isoform 2 (identifier: Q3UDW8-2) [UniParc]FASTAAdd to Basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-32: Missing.

Show »
Length:624
Mass (Da):69,100
Checksum:i5272EA184EB5BDA4
GO

Sequence cautioni

The sequence AAH24084.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
The sequence BAC29006.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
The sequence BAE31601.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
The sequence BAE35261.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
The sequence BAE35603.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 3232Missing in isoform 2. VSP_040505Add
BLAST

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti23 – 231R → H in AAH24084. 1 Publication
Sequence conflicti24 – 241N → S in AAH24084. 1 Publication
Sequence conflicti222 – 2221T → A in AAH24084. 1 Publication
Sequence conflicti242 – 2421A → G in BAE35261. 1 Publication
Sequence conflicti329 – 3291L → F in BAE29143. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AK035264 mRNA. Translation: BAC29006.1. Different initiation.
AK149883 mRNA. Translation: BAE29143.1.
AK152926 mRNA. Translation: BAE31601.1. Different initiation.
AK159649 mRNA. Translation: BAE35261.1. Different initiation.
AK160068 mRNA. Translation: BAE35603.1. Different initiation.
AC093366 Genomic DNA. No translation available.
BC024084 mRNA. Translation: AAH24084.1. Different initiation.
CCDSiCCDS40309.1. [Q3UDW8-1]
RefSeqiNP_084160.1. NM_029884.1. [Q3UDW8-1]
UniGeneiMm.28326.

Genome annotation databases

EnsembliENSMUST00000037609; ENSMUSP00000040356; ENSMUSG00000037260. [Q3UDW8-1]
GeneIDi52120.
KEGGimmu:52120.
UCSCiuc009lhg.1. mouse. [Q3UDW8-1]

Keywords - Coding sequence diversityi

Alternative initiation

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AK035264 mRNA. Translation: BAC29006.1 . Different initiation.
AK149883 mRNA. Translation: BAE29143.1 .
AK152926 mRNA. Translation: BAE31601.1 . Different initiation.
AK159649 mRNA. Translation: BAE35261.1 . Different initiation.
AK160068 mRNA. Translation: BAE35603.1 . Different initiation.
AC093366 Genomic DNA. No translation available.
BC024084 mRNA. Translation: AAH24084.1 . Different initiation.
CCDSi CCDS40309.1. [Q3UDW8-1 ]
RefSeqi NP_084160.1. NM_029884.1. [Q3UDW8-1 ]
UniGenei Mm.28326.

3D structure databases

ProteinModelPortali Q3UDW8.
ModBasei Search...
MobiDBi Search...

PTM databases

PhosphoSitei Q3UDW8.

Proteomic databases

PaxDbi Q3UDW8.
PRIDEi Q3UDW8.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000037609 ; ENSMUSP00000040356 ; ENSMUSG00000037260 . [Q3UDW8-1 ]
GeneIDi 52120.
KEGGi mmu:52120.
UCSCi uc009lhg.1. mouse. [Q3UDW8-1 ]

Organism-specific databases

CTDi 138050.
MGIi MGI:1196297. Hgsnat.

Phylogenomic databases

eggNOGi COG4299.
GeneTreei ENSGT00390000001491.
HOGENOMi HOG000006803.
HOVERGENi HBG081599.
InParanoidi Q3UDW8.
KOi K10532.
OMAi NSERCYH.
OrthoDBi EOG7PS1F9.
TreeFami TF324790.

Enzyme and pathway databases

Reactomei REACT_198967. HS-GAG degradation.

Miscellaneous databases

ChiTaRSi HGSNAT. mouse.
NextBioi 308520.
PROi Q3UDW8.
SOURCEi Search...

Gene expression databases

Bgeei Q3UDW8.
CleanExi MM_HGSNAT.
Genevestigatori Q3UDW8.

Family and domain databases

InterProi IPR012429. DUF1624.
[Graphical view ]
Pfami PF07786. DUF1624. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "The transcriptional landscape of the mammalian genome."
    Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
    , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
    Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1), NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1/2).
    Strain: C57BL/6J.
    Tissue: Bone marrow and Urinary bladder.
  2. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    Strain: C57BL/6J.
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 21-656 (ISOFORM 1/2).
    Strain: FVB/N.
    Tissue: Liver.
  4. "Identification of the gene encoding the enzyme deficient in mucopolysaccharidosis IIIC (Sanfilippo disease type C)."
    Fan X., Zhang H., Zhang S., Bagshaw R.D., Tropak M.B., Callahan J.W., Mahuran D.J.
    Am. J. Hum. Genet. 79:738-744(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY, FUNCTION, CATALYTIC ACTIVITY, SUBCELLULAR LOCATION, TISSUE SPECIFICITY.
  5. "Quantitative time-resolved phosphoproteomic analysis of mast cell signaling."
    Cao L., Yu K., Banh C., Nguyen V., Ritz A., Raphael B.J., Kawakami Y., Kawakami T., Salomon A.R.
    J. Immunol. 179:5864-5876(2007) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT TYR-249, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Mast cell.
  6. "Mass-spectrometric identification and relative quantification of N-linked cell surface glycoproteins."
    Wollscheid B., Bausch-Fluck D., Henderson C., O'Brien R., Bibel M., Schiess R., Aebersold R., Watts J.D.
    Nat. Biotechnol. 27:378-386(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-157.

Entry informationi

Entry nameiHGNAT_MOUSE
AccessioniPrimary (citable) accession number: Q3UDW8
Secondary accession number(s): E9QNP9
, Q3TWK5, Q8CBU7, Q8CIE1
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 23, 2007
Last sequence update: July 27, 2011
Last modified: September 3, 2014
This is version 71 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Miscellaneous

A signal sequence is predicted but has been shown not to be cleaved in the reticulum endoplasmic By similarity.

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot

External Data

Dasty 3

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