id: Pfam:PF01871
pfam_id: PF01871
name: AMMECR1
short_name: AMMECR1
seed_status: EX_DUF
characterization_status: UNSCORED
curation_status: SEEDED
source_url: https://www.ebi.ac.uk/interpro/api/entry/pfam/PF01871
counters:
  proteins: 7217
  matches: 7346
  proteomes: 5587
  taxa: 13329
  structures: 3
  alphafold_models: 6109
  domain_architectures: 58
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:IPR002733
description: This domain is found at the C-terminal of several AMMECR1 proteins. Homologues appear in
  species ranging from bacteria and archaea to eukaryotes, including Protein PH0010 from Pyrococcus horikoshii
  [[cite:PUB00017067]]. The high level of conservation of the AMMECR1 domain points to a basic cellular
  function, potentially in either the transcription, replication, repair or translation machinery [[cite:PUB00006433]].
  The AMMECR1 domain consists of two distinct subdomains of different sizes. The large subdomain, which
  contains both the N- and C-terminal regions, consists of five alpha-helices and five beta-strands. These
  five beta-strands form an antiparallel beta-sheet. The small subdomain consists of four alpha-helices
  and three beta-strands, and these beta-strands also form an antiparallel beta-sheet. The conserved 'LRGCIG'
  motif is located at beta(2) and its N-terminal loop, and most of the side chains of these residues point
  toward the interface of the two subdomains. The two subdomains are connected by only two loops, and
  the interaction between the two subdomains is not strong. Thus, these subdomains may move dynamically
  when the substrate enters the cleft. The size of the cleft suggests that the substrate is large, e.g.,
  the substrate may be a nucleic acid or protein. However, the inner side of the cleft is not filled with
  positively charged residues, and therefore it is unlikely that negatively charged nucleic acids such
  as DNA or RNA interact at this site [[cite:PUB00017067]]. The contiguous gene deletion syndrome AMME
  is characterised by Alport syndrome, midface hypoplasia, mental retardation and elliptocytosis and is
  caused by a deletion in Xq22.3, comprising several genes including COL4A5, FACL4 and AMMECR1 [[cite:PUB00015590]].