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Family Report for: PGAP1


Family PGAP1
Block X
Post-GPI (Glycophosphatidylinositol; Glycosylphosphatidylinositol) attachment to proteins factor 1. The sequences found in this family are similar to PGAP1 (Q765A7_RAT). This is an endoplasmic reticulum membrane protein with a catalytic serine containing motif that is conserved in a number of lipases. PGAP1 functions as a GPI inositol-deacylase; this deacylation is important for the efficient transport of GPI-anchored proteins from the endoplasmic reticulum to the Golgi body. This family consists of several hypothetical bacterial proteins of unknown function. Members of this family have an alpha/beta hydrolase fold. The type VI lipase effectors Tle1-Tle5 secreted by the bacterial type VI secretion system (T6SS) have recently been identified as antibacterial effectors that hydrolyze membrane phospholipids. The Tle1-4 display phospholipase A1 and A2 (PLA1) (PLA2) activities. Tle1 belongs to Duf_2235 family (now T6SS-TLE1), TLE2 to Lipase_3, TLE3 to alpha/ beta hydrolase (now T6SS-TLE3) and here Tle4 belongs to PGAP1. There are two human genes in this family. One is SERAC1. Mutations of SERAC1 causes MEGDEL syndrome, a recessive disorder of dystonia and deafness with encephalopathy and Leigh-like syndrome, impaired oxidative phosphorylation and 3-methylglutaconic aciduria. Clinical features included psychomotor retardation, recurrent infections in infancy, hypoglycemia, spasticity, dystonia, sensorineural deafness, brain atrophy, and lesions on brain imaging. Laboratory studies showed increased serum lactate and alanine, urinary 3-MGA, mitochondrial oxidative phosphorylation defects, abnormal mitochondria, an abnormal phosphatidylglycerol and cardiolipin spectrum in fibroblasts, and abnormal accumulation of unesterified cholesterol within cells.The other is PGAP1 Mutations in PGAP1 causes Mental retardation, autosomal recessive 42 MRT42
2 Disease(s)
MEGDEL syndrome from mutation R148X_human-SERAC1 in gene_locus human-SERAC1.

IPR012908 (GPI inositol-deacylase PGAP1-like), IPR039529 (GPI inositol-deacylase)
PF07819 (PGAP1 GPI inositol-deacylase)
no EC number

Peptide in
Nucleotide in
Alignment with Multalin
|Text only/graphic display
Seed alignment with MAFFT
|No colour/coloured with Mview
Alignment with MAFFT
|No colour/coloured with Mview
|Graphical display, obtained with the dnd file produced by Clustalw

1 more
    Title: The structural basis of the Tle4-Tli4 complex reveals the self-protection mechanism of H2-T6SS in Pseudomonas aeruginosa
    Lu D, Zheng Y, Liao N, Wei L, Xu B, Liu X, Liu J
    Ref: Acta Crystallographica D Biol Crystallogr, 70:3233, 2014 : PubMed


    Title: Null mutation in PGAP1 impairing Gpi-anchor maturation in patients with intellectual disability and encephalopathy
    Murakami Y, Tawamie H, Maeda Y, Buttner C, Buchert R, Radwan F, Schaffer S, Sticht H, Aigner M and Jamra RA <2 more author(s)>
    Ref: PLoS Genet, 10:e1004320, 2014 : PubMed


    Title: Inositol deacylation of glycosylphosphatidylinositol-anchored proteins is mediated by mammalian PGAP1 and yeast Bst1p
    Tanaka S, Maeda Y, Tashima Y, Kinoshita T
    Ref: Journal of Biological Chemistry, 279:14256, 2004 : PubMed


Other Papers

No structure scheme yet for this family

Structures in PGAP1 family (2)

Genes Proteins in PGAP1 family (156)

Fragments of genes in PGAP1 family (2)

Substrates of some enzymes in the PGAP1 family (1)

Inhibitors of some enzymes in the PGAP1 family (3)

Send your questions or comments to :
Mail to: Nicolas Lenfant, Thierry Hotelier, Yves Bourne, Pascale Marchot and Arnaud Chatonnet.
Please cite: Lenfant 2013 Nucleic.Acids.Res. or Marchot Chatonnet 2012 Prot.Pept Lett.
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