Family: PGAP1

Block: X

Parent Family: No family


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


Interpro : IPR012908 GPI inositol-deacylase PGAP1-like , IPR039529 GPI inositol-deacylase


Pdoc : No Pdoc

Pfam : PF07819 PGAP1 GPI inositol-deacylase

Prints : No Print

EC Number : No EC Number


Peptide in Fasta
Nucleotide in Fasta
Alignment with Multalin Text only
Seed alignment with MAFFT No colour
Alignment with MAFFT No colour
Dendrogram The dnd file

Structures (4)

Genes Proteins in PGAP1 family (156)

Fragments of genes in PGAP1 family (2)

Structures in PGAP1 family (4)

Substrates in PGAP1 family (1)

Inhibitors in PGAP1 family (5)

References (5)

Title : Molecular basis of the inositol deacylase PGAP1 involved in quality control of GPI-AP biogenesis - Hong_2024_Nat.Commun_15_8
Author(s) : Hong J , Li T , Chao Y , Xu Y , Zhu Z , Zhou Z , Gu W , Qu Q , Li D
Ref : Nat Commun , 15 :8 , 2024
PubMedID: 38167496
Gene_locus related to this paper: chatd-g0s652 , human-PGAP1

Title : Null mutation in PGAP1 impairing Gpi-anchor maturation in patients with intellectual disability and encephalopathy - Murakami_2014_PLoS.Genet_10_e1004320
Author(s) : Murakami Y , Tawamie H , Maeda Y , Buttner C , Buchert R , Radwan F , Schaffer S , Sticht H , Aigner M , Reis A , Kinoshita T , Jamra RA
Ref : PLoS Genet , 10 :e1004320 , 2014
PubMedID: 24784135
Gene_locus related to this paper: human-PGAP1

Title : The structural basis of the Tle4-Tli4 complex reveals the self-protection mechanism of H2-T6SS in Pseudomonas aeruginosa - Lu_2014_Acta.Crystallogr.D.Biol.Crystallogr_70_3233
Author(s) : Lu D , Zheng Y , Liao N , Wei L , Xu B , Liu X , Liu J
Ref : Acta Crystallographica D Biol Crystallogr , 70 :3233 , 2014
PubMedID: 25478841
Gene_locus related to this paper: pseae-PA1510

Title : Mutations in the phospholipid remodeling gene SERAC1 impair mitochondrial function and intracellular cholesterol trafficking and cause dystonia and deafness - Wortmann_2012_Nat.Genet_44_797
Author(s) : Wortmann SB , Vaz FM , Gardeitchik T , Vissers LE , Renkema GH , Schuurs-Hoeijmakers JH , Kulik W , Lammens M , Christin C , Kluijtmans LA , Rodenburg RJ , Nijtmans LG , Grunewald A , Klein C , Gerhold JM , Kozicz T , van Hasselt PM , Harakalova M , Kloosterman W , Baric I , Pronicka E , Ucar SK , Naess K , Singhal KK , Krumina Z , Gilissen C , van Bokhoven H , Veltman JA , Smeitink JA , Lefeber DJ , Spelbrink JN , Wevers RA , Morava E , de Brouwer AP
Ref : Nat Genet , 44 :797 , 2012
PubMedID: 22683713
Gene_locus related to this paper: human-SERAC1

Title : Inositol deacylation of glycosylphosphatidylinositol-anchored proteins is mediated by mammalian PGAP1 and yeast Bst1p - Tanaka_2004_J.Biol.Chem_279_14256
Author(s) : Tanaka S , Maeda Y , Tashima Y , Kinoshita T
Ref : Journal of Biological Chemistry , 279 :14256 , 2004
PubMedID: 14734546
Gene_locus related to this paper: human-PGAP1 , ratno-q765a7 , yeast-BST1