› 6v7n

X-ray diffraction
2.62Å resolution

Crystal Structure of a human Lysosome Resident Glycoprotein, Lysosomal Acid Lipase, and its Implications in Cholesteryl Ester Storage Disease (CESD)

Released:
DOI: 10.2210/pdb6v7n/pdb
PDB entry 6v7n coloured by chain, front view.
PDB entry 6v7n coloured by chain, side view.
PDB entry 6v7n coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Crystal structure of human lysosomal acid lipase and its implications in cholesteryl ester storage disease.
Rajamohan F, Reyes AR, Tu M, Nedoma NL, Hoth LR, Schwaid AG, Kurumbail RG, Ward J, Han S
J Lipid Res 61 1192-1202 (2020)
PMID: 32482718

Function and Biology Details

Reaction catalysed: 
A steryl ester + H(2)O = a sterol + a fatty acid
Biochemical function:
Biological process:
  • not assigned
Cellular component:
  • not assigned
Sequence domains:

Structure analysis Details

Assembly composition:
monomeric (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-153680 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (2 distinct):
Lysosomal acid lipase/cholesteryl ester hydrolase Chains: A, B
Molecule details ›
Chains: A, B
Length: 384 amino acids
Theoretical weight: 43.72 KDa
Source organism: Homo sapiens
Expression system: Spodoptera frugiperda
UniProt:
  • Canonical: P38571 (Residues: 22-399; Coverage: 100%)
Gene name: LIPA
Sequence domains: alpha/beta hydrolase fold

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
2 bound ligands:
Ligand NAG 3 x NAG
Ligand SO4 3 x SO4
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 17-ID
Spacegroup: P21212
Unit cell:
a: 97.145Å b: 165.486Å c: 60.36Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.187 0.185 0.222
Expression system: Spodoptera frugiperda

Quick links

Citations

3 review citations

Lipolysis: cellular mechanisms for lipid mobilization from fat stores.
Grabner et al. (2021)
2 more

5 mentions without citation

Recent insights into lysosomal acid lipase deficiency.
Korbelius et al. (2023)
4 more