id: Pfam:PF04685
pfam_id: PF04685
name: Glycosyl-hydrolase family 116, catalytic region
short_name: GH116_catalytic
seed_status: EX_DUF
characterization_status: UNSCORED
curation_status: SEEDED
source_url: https://www.ebi.ac.uk/interpro/api/entry/pfam/PF04685
counters:
  proteins: 5324
  matches: 5493
  proteomes: 2539
  taxa: 6811
  structures: 36
  alphafold_models: 4187
  domain_architectures: 83
provenance:
  snapshot_id: interpro-pfam-duf-2026-10-10
  path: data/worklists/interpro-pfam-duf-2026-10-10.json
  sha256: 17fb625876c9326ed284263a47bcd84e0d7844a31c040dc06cd2260c36f1c383
  generated_at: '2026-10-10T07:44:21.614711Z'
  source_url: https://www.ebi.ac.uk/interpro/api/entry/pfam/
interpro_id: InterPro:IPR006775
description: This represents a family of archaeal, bacterial and eukaryotic glycosyl hydrolases, that
  belong to superfamily GH116. The primary catabolic pathway for glucosylceramide is catalysis by the
  lysosomal enzyme glucocerebrosidase. In higher eukaryotes, glucosylceramide is the precursor of glycosphingolipids,
  a complex group of ubiquitous membrane lipids [[cite:PUB00044658]]. Mutations in the human protein cause
  motor-neurone defects in hereditary spastic paraplegia [[cite:PUB00078737]]. The catalytic nucleophile,
  identified in UniProtKB:Q97YG8_SULSO, is a glutamine-335, with the likely acid/base at Asp-442 and the
  aspartates at Asp-406 and Asp-458 residues also playing a role in the catalysis of glucosides and xylosides
  that are beta-bound to hydrophobic groups. The family is defined as GH116, which presently includes
  enzymes with beta-glucosidase, [ec:3.2.1.21], beta-xylosidase, [ec:3.2.1.37], and glucocerebrosidase
  [ec:3.2.1.45] activity [[cite:PUB00044658],[cite:PUB00073615]].