› 6o4e

X-ray diffraction
1.75Å resolution

Structure of ALDH7A1 mutant N167S complexed with NAD

Released:
DOI: 10.2210/pdb6o4e/pdb
PDB entry 6o4e coloured by chain, front view.
PDB entry 6o4e coloured by chain, side view.
PDB entry 6o4e coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Structural and biochemical consequences of pyridoxine-dependent epilepsy mutations that target the aldehyde binding site of aldehyde dehydrogenase ALDH7A1.
Laciak AR, Korasick DA, Wyatt JW, Gates KS, Tanner JJ
FEBS J 287 173-189 (2020)
PMID: 31302938

Function and Biology Details

Reactions catalysed: 
An aldehyde + NAD(+) + H(2)O = a carboxylate + NADH
(1a) (S)-2-amino-6-oxohexanoate = (S)-2,3,4,5-tetrahydropyridine-2-carboxylate + H(2)O (spontaneous)
Betaine aldehyde + NAD(+) + H(2)O = betaine + NADH
Biochemical function:
Biological process:
Cellular component:
Sequence domains:

Structure analysis Details

Assembly composition:
homo tetramer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-155949 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Alpha-aminoadipic semialdehyde dehydrogenase Chains: A, B, C, D, E, F, G, H
Molecule details ›
Chains: A, B, C, D, E, F, G, H
Length: 513 amino acids
Theoretical weight: 55.59 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli
UniProt:
  • Canonical: P49419 (Residues: 29-539; Coverage: 95%)
Gene names: ALDH7A1, ATQ1
Sequence domains: Aldehyde dehydrogenase family

Ligands and Environments


Cofactor: Ligand NAD 8 x NAD
4 bound ligands:
Ligand PG4 3 x PG4
Ligand 1PE 4 x 1PE
Ligand MG 6 x MG
Ligand P6G 1 x P6G
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: ALS BEAMLINE 4.2.2
Spacegroup: C2
Unit cell:
a: 154.92Å b: 161.73Å c: 158.41Å
α: 90° β: 94.65° γ: 90°
R-values:
R R work R free
0.17 0.168 0.202
Expression system: Escherichia coli

Quick links

Citations

1 review citation

Impact of missense mutations in the ALDH7A1 gene on enzyme structure and catalytic function.
Korasick et al. (2021)
2 other articles

2 mentions without citation

Structural analysis of pathogenic mutations targeting Glu427 of ALDH7A1, the hot spot residue of pyridoxine-dependent epilepsy.
Laciak et al. (2020)
1 more