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3. Structural and mechanistic insights into a lysosomal membrane enzyme HGSNAT involved in Sanfilippo syndrome. Zhao B; Cao Z; Zheng Y; Nguyen P; Bowen A; Edwards RH; Stroud RM; Zhou Y; Van Lookeren Campagne M; Li F Nat Commun; 2024 Jun; 15(1):5388. PubMed ID: 38918376 [TBL] [Abstract][Full Text] [Related]
4. Sanfilippo syndrome type C: deficiency of acetyl-CoA:alpha-glucosaminide N-acetyltransferase in skin fibroblasts. Klein U; Kresse H; von Figura K Proc Natl Acad Sci U S A; 1978 Oct; 75(10):5185-9. PubMed ID: 33384 [TBL] [Abstract][Full Text] [Related]
5. Analysis of the biogenesis of heparan sulfate acetyl-CoA:alpha-glucosaminide N-acetyltransferase provides insights into the mechanism underlying its complete deficiency in mucopolysaccharidosis IIIC. Durand S; Feldhammer M; Bonneil E; Thibault P; Pshezhetsky AV J Biol Chem; 2010 Oct; 285(41):31233-42. PubMed ID: 20650889 [TBL] [Abstract][Full Text] [Related]
6. Sanfilippo syndrome type C: assay for acetyl-CoA: alpha-glucosaminide N-acetyltransferase in leukocytes for detection of homozygous and heterozygous individuals. Klein U; van de Kamp JJ; von Figura K; Pohlmann R Clin Genet; 1981 Jul; 20(1):55-9. PubMed ID: 6794963 [TBL] [Abstract][Full Text] [Related]
8. Acetyl-coenzyme A:alpha-glucosaminide N-acetyltransferase. Evidence for an active site histidine residue. Bame KJ; Rome LH J Biol Chem; 1986 Aug; 261(22):10127-32. PubMed ID: 3733705 [TBL] [Abstract][Full Text] [Related]
9. The diagnosis of the Sanfilippo C syndrome, using monosaccharide and oligosaccharide substrates to assay acetyl-CoA: 2-amino-2-deoxy-alpha-glucoside N-acetyltransferase activity. Hopwood JJ; Elliott H Clin Chim Acta; 1981 Apr; 112(1):67-75. PubMed ID: 6786804 [TBL] [Abstract][Full Text] [Related]
10. Sanfilippo syndrome type C: mutation spectrum in the heparan sulfate acetyl-CoA: alpha-glucosaminide N-acetyltransferase (HGSNAT) gene. Feldhammer M; Durand S; Mrázová L; Boucher RM; Laframboise R; Steinfeld R; Wraith JE; Michelakakis H; van Diggelen OP; Hrebícek M; Kmoch S; Pshezhetsky AV Hum Mutat; 2009 Jun; 30(6):918-25. PubMed ID: 19479962 [TBL] [Abstract][Full Text] [Related]
11. Structure and mechanism of lysosome transmembrane acetylation by HGSNAT. Xu R; Ning Y; Ren F; Gu C; Zhu Z; Pan X; Pshezhetsky AV; Ge J; Yu J Nat Struct Mol Biol; 2024 Oct; 31(10):1502-1508. PubMed ID: 38769387 [TBL] [Abstract][Full Text] [Related]
12. An acetylated 120-kDa lysosomal transmembrane protein is absent from mucopolysaccharidosis IIIC fibroblasts: a candidate molecule for MPS IIIC. Ausseil J; Landry K; Seyrantepe V; Trudel S; Mazur A; Lapointe F; Pshezhetsky AV Mol Genet Metab; 2006 Jan; 87(1):22-31. PubMed ID: 16293432 [TBL] [Abstract][Full Text] [Related]
13. A 3H-labelled trisaccharide from heparin as substrate for acetyl-CoA: 2-amino-2-deoxy-alpha-D-glucoside N-acetyltransferase. Klein U; von Figura K Carbohydr Res; 1980 Jan; 78(2):249-56. PubMed ID: 6766355 [TBL] [Abstract][Full Text] [Related]
14. HGSNAT enzyme deficiency results in accumulation of heparan sulfate in podocytes and basement membranes. Nagel L; Oliveira R; Pshezhetsky AV; Morales CR Histol Histopathol; 2019 Dec; 34(12):1377-1385. PubMed ID: 31157913 [TBL] [Abstract][Full Text] [Related]
15. Structure of the human heparan-α-glucosaminide Navratna V; Kumar A; Rana JK; Mosalaganti S Elife; 2024 Aug; 13():. PubMed ID: 39196614 [TBL] [Abstract][Full Text] [Related]
16. Sanfilippo disease type D: deficiency of N-acetylglucosamine-6-sulfate sulfatase required for heparan sulfate degradation. Kresse H; Paschke E; von Figura K; Gilberg W; Fuchs W Proc Natl Acad Sci U S A; 1980 Nov; 77(11):6822-6. PubMed ID: 6450420 [TBL] [Abstract][Full Text] [Related]
17. Sanfilippo type C diagnosis: assay of acetyl-CoA: alpha-glucosaminide N-acetyltransferase using [14C]glucosamine as substrate and leukocytes as enzyme source. Pallini R; Leder IG; di Natale P Pediatr Res; 1984 Jun; 18(6):543-5. PubMed ID: 6429622 [TBL] [Abstract][Full Text] [Related]
18. Functional analysis of the HGSNAT gene in patients with mucopolysaccharidosis IIIC (Sanfilippo C Syndrome). Fedele AO; Hopwood JJ Hum Mutat; 2010 Jul; 31(7):E1574-86. PubMed ID: 20583299 [TBL] [Abstract][Full Text] [Related]
19. Cellular location of N-acetyltransfer activities toward glucosamine and glucosamine-6-phosphate in cultured human skin fibroblasts. Hopwood JJ; Freeman C; Clements PR; Stein R; Miller AL Biochem Int; 1983 Jun; 6(6):823-30. PubMed ID: 6679737 [TBL] [Abstract][Full Text] [Related]
20. Lysosomal storage of heparan sulfate causes mitochondrial defects, altered autophagy, and neuronal death in the mouse model of mucopolysaccharidosis III type C. Pshezhetsky AV Autophagy; 2016 Jun; 12(6):1059-60. PubMed ID: 25998837 [TBL] [Abstract][Full Text] [Related] [Next] [New Search]