id: Pfam:PF01256
pfam_id: PF01256
name: NAD(P)H-hydrate dehydratase
short_name: Carb_kinase
seed_status: EX_DUF
characterization_status: UNSCORED
curation_status: SEEDED
source_url: https://www.ebi.ac.uk/interpro/api/entry/pfam/PF01256
counters:
  proteins: 22302
  matches: 22644
  proteomes: 19898
  taxa: 32358
  structures: 37
  alphafold_models: 15274
  domain_architectures: 88
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:IPR000631
description: Hydration of NAD(P)H to NAD(P)HX, which inhibits several dehydrogenases, is corrected by
  an ATP-dependent dehydratase and an epimerase. The ATP-dependent dehydratase has been identified as
  the product of the vertebrate Carkd (carbohydrate kinase domain) gene [[cite:PUB00073185],[cite:PUB00100876]].
  Variants of this enzyme leads to deficient NADHX repair mitochondrial dysfunction in humans, associated
  with a lethal neurometabolic disorder in early childhood [[cite:PUB00100876]]. In E. coli, it is found
  as the C-terminal domain of a bifunctional enzyme (YjeF) that also includes the epimerase and uses ADP
  instead of ATP [[cite:PUB00060794]]. These enzymes are widespread in eukaryotes, prokaryotes, and archaea.