Alpha-N-acetylgalactosaminidase

NameAlpha-N-acetylgalactosaminidase
Synonyms
  • 3.2.1.49
  • Alpha-galactosidase B
Gene NameNAGA
OrganismHuman
Amino acid sequence
>lcl|BSEQ0009538|Alpha-N-acetylgalactosaminidase
MLLKTVLLLGHVAQVLMLDNGLLQTPPMGWLAWERFRCNINCDEDPKNCISEQLFMEMAD
RMAQDGWRDMGYTYLNIDDCWIGGRDASGRLMPDPKRFPHGIPFLADYVHSLGLKLGIYA
DMGNFTCMGYPGTTLDKVVQDAQTFAEWKVDMLKLDGCFSTPEERAQGYPKMAAALNATG
RPIAFSCSWPAYEGGLPPRVNYSLLADICNLWRNYDDIQDSWWSVLSILNWFVEHQDILQ
PVAGPGHWNDPDMLLIGNFGLSLEQSRAQMALWTVLAAPLLMSTDLRTISAQNMDILQNP
LMIKINQDPLGIQGRRIHKEKSLIEVYMRPLSNKASALVFFSCRTDMPYRYHSSLGQLNF
TGSVIYEAQDVYSGDIISGLRDETNFTVIINPSGVVMWYLYPIKNLEMSQQ
Number of residues411
Molecular Weight46564.15
Theoretical pINot Available
GO Classification
Functions
  • alpha-N-acetylgalactosaminidase activity
  • protein homodimerization activity
  • alpha-galactosidase activity
Processes
  • carbohydrate catabolic process
  • glycolipid catabolic process
  • glycoside catabolic process
  • glycosylceramide catabolic process
  • oligosaccharide metabolic process
Components
  • cytoplasm
  • lysosome
  • extracellular exosome
General FunctionProtein homodimerization activity
Specific FunctionRemoves terminal alpha-N-acetylgalactosamine residues from glycolipids and glycopeptides. Required for the breakdown of glycolipids.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein IDNot Available
UniProtKB IDP17050
UniProtKB Entry NameNAGAB_HUMAN
Cellular LocationLysosome
Gene sequence
>lcl|BSEQ0020779|Alpha-N-acetylgalactosaminidase (NAGA)
ATGCTGCTGAAGACAGTGCTCTTGCTGGGACATGTGGCCCAGGTGCTGATGCTGGACAAT
GGGCTCCTGCAGACACCACCCATGGGCTGGCTGGCCTGGGAACGCTTCCGCTGCAACATT
AACTGTGATGAGGACCCAAAGAACTGCATAAGTGAACAGCTCTTCATGGAGATGGCTGAC
CGGATGGCACAGGATGGATGGCGGGACATGGGCTACACATACCTCAACATTGATGACTGC
TGGATCGGTGGTCGCGATGCCAGTGGCCGCCTGATGCCGGATCCCAAGCGCTTCCCTCAT
GGCATTCCTTTCCTGGCTGACTACGTTCACTCCCTGGGCCTGAAGTTGGGTATCTACGCG
GACATGGGCAACTTCACCTGCATGGGTTACCCAGGCACCACACTGGACAAGGTGGTCCAG
GATGCTCAGACCTTCGCCGAGTGGAAGGTAGACATGCTCAAGCTGGATGGCTGCTTCTCC
ACCCCCGAGGAGCGGGCCCAGGGGTACCCCAAGATGGCTGCTGCCCTGAATGCCACAGGC
CGCCCCATCGCCTTCTCCTGCAGCTGGCCAGCCTATGAAGGCGGCCTCCCCCCAAGGGTG
AACTACAGTCTGCTGGCGGACATCTGCAACCTCTGGCGTAACTATGATGACATCCAGGAC
TCCTGGTGGAGCGTGCTCTCCATCCTGAATTGGTTCGTGGAGCACCAGGACATACTGCAG
CCAGTGGCCGGCCCTGGGCACTGGAATGACCCTGACATGCTGCTCATTGGGAACTTTGGT
CTCAGCTTAGAGCAATCCCGGGCCCAGATGGCCCTGTGGACGGTGCTGGCAGCCCCCCTC
TTGATGTCCACAGACCTGCGTACCATCTCCGCCCAGAACATGGACATTCTGCAGAATCCA
CTCATGATCAAAATCAACCAGGATCCCTTAGGCATCCAGGGACGCAGGATTCACAAGGAA
AAATCTCTCATCGAAGTGTACATGCGGCCTCTGTCCAACAAGGCTAGCGCCTTAGTCTTC
TTCAGCTGCAGGACCGATATGCCTTATCGCTACCACTCCTCCCTTGGCCAGCTGAACTTC
ACCGGGTCTGTGATATATGAGGCCCAGGACGTCTACTCAGGTGACATCATCAGTGGCCTC
CGAGATGAAACCAACTTCACAGTGATCATCAACCCTTCAGGGGTAGTGATGTGGTACCTG
TATCCCATCAAGAACCTGGAGATGTCCCAGCAGTGA
GenBank Gene IDNot Available
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:7631
Chromosome Location22
LocusNot Available
References
  1. Wang AM, Bishop DF, Desnick RJ: Human alpha-N-acetylgalactosaminidase-molecular cloning, nucleotide sequence, and expression of a full-length cDNA. Homology with human alpha-galactosidase A suggests evolution from a common ancestral gene. J Biol Chem. 1990 Dec 15;265(35):21859-66. 2174888
  2. Wang AM, Desnick RJ: Structural organization and complete sequence of the human alpha-N-acetylgalactosaminidase gene: homology with the alpha-galactosidase A gene provides evidence for evolution from a common ancestral gene. Genomics. 1991 May;10(1):133-42. 1646157
  3. Tsuji S, Yamauchi T, Hiraiwa M, Isobe T, Okuyama T, Sakimura K, Takahashi Y, Nishizawa M, Uda Y, Miyatake T: Molecular cloning of a full-length cDNA for human alpha-N-acetylgalactosaminidase (alpha-galactosidase B). Biochem Biophys Res Commun. 1989 Sep 29;163(3):1498-504. 2551294
  4. Yamauchi T, Hiraiwa M, Kobayashi H, Uda Y, Miyatake T, Tsuji S: Molecular cloning of two species of cDNAs for human alpha-N-acetylgalactosaminidase and expression in mammalian cells. Biochem Biophys Res Commun. 1990 Jul 16;170(1):231-7. 2372288
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  9. Asfaw B, Schindler D, Ledvinova J, Cerny B, Smid F, Conzelmann E: Degradation of blood group A glycolipid A-6-2 by normal and mutant human skin fibroblasts. J Lipid Res. 1998 Sep;39(9):1768-80. 9741689
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  15. Wang AM, Kanzaki T, Desnick RJ: The molecular lesion in the alpha-N-acetylgalactosaminidase gene that causes angiokeratoma corporis diffusum with glycopeptiduria. J Clin Invest. 1994 Aug;94(2):839-45. 8040340
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