UniProt Accession

 AGAL_HUMAN; P06280 

Genbank Protein ID

 X05790; X14448; U78027; AL035422; BC002689; M13571; D00039; M18242; X16889; M20317 

Genbank Nucleotide ID

 CAA29232.1; CAA32617.1; AAB64203.1; CAB55878.1; AAH02689.1; AAA51676.1; AAA52514.1; AAA52559.1 

Protein Name

 Alpha-galactosidase A 

Protein Synonyms/Alias

 Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; Agalsidase 

Gene Name

 GLA 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Homo sapiens (Human) 

NCBI Taxa ID

 9606 

Lysine Modification

Position	Peptide	Type	References
185	ENLADGYKHMSLALN	ubiquitination	[1]
237	ADIDDSWKSIKSILD	ubiquitination	[2, 3, 4]
240	DDSWKSIKSILDWTS	ubiquitination	[1, 2, 3, 4, 5, 6, 7]
308	RHISPQAKALLQDKD	ubiquitination	[1, 2]
314	AKALLQDKDVIAINQ	ubiquitination	[1, 2, 3]
326	INQDPLGKQGYQLRQ	ubiquitination	[1, 2, 3, 4, 5, 7]
391	ITQLLPVKRKLGFYE	ubiquitination	[1, 3]
393	QLLPVKRKLGFYEWT	ubiquitination	[3, 5, 7]

Reference

 [1] Global identification of modular cullin-RING ligase substrates.
 Emanuele MJ, Elia AE, Xu Q, Thoma CR, Izhar L, Leng Y, Guo A, Chen YN, Rush J, Hsu PW, Yen HC, Elledge SJ.
 Cell. 2011 Oct 14;147(2):459-74. [PMID: 21963094]
 [2] Systematic and quantitative assessment of the ubiquitin-modified proteome.
 Kim W, Bennett EJ, Huttlin EL, Guo A, Li J, Possemato A, Sowa ME, Rad R, Rush J, Comb MJ, Harper JW, Gygi SP.
 Mol Cell. 2011 Oct 21;44(2):325-40. [PMID: 21906983]
 [3] Refined preparation and use of anti-diglycine remnant (K-ε-GG) antibody enables routine quantification of 10,000s of ubiquitination sites in single proteomics experiments.
 Udeshi ND, Svinkina T, Mertins P, Kuhn E, Mani DR, Qiao JW, Carr SA.
 Mol Cell Proteomics. 2013 Mar;12(3):825-31. [PMID: 23266961]
 [4] Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization.
 Sarraf SA, Raman M, Guarani-Pereira V, Sowa ME, Huttlin EL, Gygi SP, Harper JW.
 Nature. 2013 Apr 18;496(7445):372-6. [PMID: 23503661]
 [5] A proteome-wide, quantitative survey of in vivo ubiquitylation sites reveals widespread regulatory roles.
 Wagner SA, Beli P, Weinert BT, Nielsen ML, Cox J, Mann M, Choudhary C.
 Mol Cell Proteomics. 2011 Oct;10(10):M111.013284. [PMID: 21890473]
 [6] Ubiquitin ligase substrate identification through quantitative proteomics at both the protein and peptide levels.
 Lee KA, Hammerle LP, Andrews PS, Stokes MP, Mustelin T, Silva JC, Black RA, Doedens JR.
 J Biol Chem. 2011 Dec 2;286(48):41530-8. [PMID: 21987572]
 [7] hCKSAAP_UbSite: improved prediction of human ubiquitination sites by exploiting amino acid pattern and properties.
 Chen Z, Zhou Y, Song J, Zhang Z.
 Biochim Biophys Acta. 2013 Aug;1834(8):1461-7. [PMID: 23603789] 

Functional Description

  

Sequence Annotation

 REGION 203 207 Substrate binding.
 ACT_SITE 170 170 Nucleophile (By similarity).
 ACT_SITE 231 231 Proton donor (By similarity).
 CARBOHYD 139 139 N-linked (GlcNAc...).
 CARBOHYD 192 192 N-linked (GlcNAc...).
 CARBOHYD 215 215 N-linked (GlcNAc...).
 CARBOHYD 408 408 N-linked (GlcNAc...) (Potential).
 DISULFID 52 94
 DISULFID 56 63
 DISULFID 142 172
 DISULFID 202 223
 DISULFID 378 382  

Keyword

 3D-structure; Complete proteome; Direct protein sequencing; Disease mutation; Disulfide bond; Glycoprotein; Glycosidase; Hydrolase; Lysosome; Pharmaceutical; Reference proteome; RNA editing; Signal. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 429 AA 

Protein Sequence

Gene Ontology

 GO:0005576; C:extracellular region; IDA:UniProtKB.
 GO:0005794; C:Golgi apparatus; IMP:UniProtKB.
 GO:0043202; C:lysosomal lumen; TAS:Reactome.
 GO:0004557; F:alpha-galactosidase activity; IDA:UniProtKB.
 GO:0016936; F:galactoside binding; IEA:Compara.
 GO:0042803; F:protein homodimerization activity; IDA:UniProtKB.
 GO:0052692; F:raffinose alpha-galactosidase activity; IEA:EC.
 GO:0005102; F:receptor binding; IDA:UniProtKB.
 GO:0016139; P:glycoside catabolic process; IBA:RefGenome.
 GO:0046477; P:glycosylceramide catabolic process; ISS:UniProtKB.
 GO:0045019; P:negative regulation of nitric oxide biosynthetic process; ISS:UniProtKB.
 GO:0051001; P:negative regulation of nitric-oxide synthase activity; ISS:UniProtKB.
 GO:0009311; P:oligosaccharide metabolic process; IDA:UniProtKB.
 GO:0044281; P:small molecule metabolic process; TAS:Reactome. 

Interpro

 IPR013785; Aldolase_TIM.
 IPR013780; Glyco_hydro_13_b.
 IPR002241; Glyco_hydro_27.
 IPR000111; Glyco_hydro_GHD.
 IPR017853; Glycoside_hydrolase_SF. 

Pfam

 PF02065; Melibiase 

SMART

  

PROSITE

 PS00512; ALPHA_GALACTOSIDASE 

PRINTS

 PR00740; GLHYDRLASE27.