9.B.169.4.1
Heparana α-glucosaminide N-acetyltransferase, HGSNAT or MEM76. Of 663 aas and 12 TMSs in a 1 + 1 + 5 + 5 TMS arrangement. Mutations cause mucopolysaccharidosis 3C (Fan et al. 2006). This lysosomal acetyltransferase 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 (Durand et al. 2010).
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| Accession Number: | Q68CP4 |
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| Protein Name: | Heparan-alpha-glucosaminide N-acetyltransferase |
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| Length: | 663 |
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| Molecular Weight: | 73293.00 |
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| Species: | Homo sapiens (Human) [9606] |
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| Number of TMSs: | 12 |
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| Location1 / Topology2 / Orientation3: |
Lysosome membrane1 / Multi-pass membrane protein2 |
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| Substrate |
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1: MTGARASAAE QRRAGRSGQA RAAERAAGMS GAGRALAALL LAASVLSAAL LAPGGSSGRD
61: AQAAPPRDLD KKRHAELKMD QALLLIHNEL LWTNLTVYWK SECCYHCLFQ VLVNVPQSPK
121: AGKPSAAAAS VSTQHGSILQ LNDTLEEKEV CRLEYRFGEF GNYSLLVKNI HNGVSEIACD
181: LAVNEDPVDS NLPVSIAFLI GLAVIIVISF LRLLLSLDDF NNWISKAISS RETDRLINSE
241: LGSPSRTDPL DGDVQPATWR LSALPPRLRS VDTFRGIALI LMVFVNYGGG KYWYFKHASW
301: NGLTVADLVF PWFVFIMGSS IFLSMTSILQ RGCSKFRLLG KIAWRSFLLI CIGIIIVNPN
361: YCLGPLSWDK VRIPGVLQRL GVTYFVVAVL ELLFAKPVPE HCASERSCLS LRDITSSWPQ
421: WLLILVLEGL WLGLTFLLPV PGCPTGYLGP GGIGDFGKYP NCTGGAAGYI DRLLLGDDHL
481: YQHPSSAVLY HTEVAYDPEG ILGTINSIVM AFLGVQAGKI LLYYKARTKD ILIRFTAWCC
541: ILGLISVALT KVSENEGFIP VNKNLWSLSY VTTLSSFAFF ILLVLYPVVD VKGLWTGTPF
601: FYPGMNSILV YVGHEVFENY FPFQWKLKDN QSHKEHLTQN IVATALWVLI AYILYRKKIF
661: WKI