Structure of PDB 4ufl Chain A

Receptor sequence

>4uflA (length=641) Species: 10090 (Mus musculus)

GAYVLDDSDGLGREFDGIGAVSGGGATSRLLVNYPEPYRSEILDYLFKPN
FGASLHILKVEIGGDGQTTDGTEPSHMHYELDENYFRGYEWWLMKEAKKR
NPDIILMGLPWSFPGWLGKGFSWPYVNLQLTAYYVVRWILGAKHYHDLDI
DYIGIWNERPFDANYIKELRKMLDYQGLQRVRIIASDNLWEPISSSLLLD
QELWKVVDVIGAHYPGTYTVWNAKMSGKKLWSSEDFSTINSNVGAGCWSR
ILNQNYINGNMTSTIAWNLVASYYEELPYGRSGLMTAQEPWSGHYVVASP
IWVSAHTTQFTQPGWYYLKTVGHLEKGGSYVALTDGLGNLTIIIETMSHQ
HSMCIRPYLPYYNVSHQLATFTLKGSLREIQELQVWYTKLGQRLHFKQLD
TLWLLDGSGSFTLELEEDEIFTLTTLTTGRKGSYPPPPSSKPFPTNYKDD
FNVEYPLFSEAPNFADQTGVFEYYMNNEDREHRFTLRQVLNQRPITWAAD
ASSTISVIGDHHWTNMTVQCDVYIETPRSGGVFIAGRVNKGGILIRSATG
VFFWIFANGSYRVTADLGGWITYASGHADVTAKRWYTLTLGIKGYFAFGM
LNGTILWKNVRVKYPGHGWAAIGTHTFEFAQFDNFRVEAAR

3D structure

• PDB: 4ufl Azasugar Inhibitors as Pharmacological Chaperones for Krabbe Disease.
• Chain: A
• Resolution: 2.4 Å

Enzymatic activity

• Enzyme Commision number: 3.2.1.46: galactosylceramidase.

Interaction with ligand

Site #	Ligand	Ligand chain	Binding residues on receptor (original residue number in PDB)	Binding residues on receptor (residue number reindexed from 1)	Binding affinity
BS01	DZX	A	G48 T93 E182 Y238 E258 S261 W291 Y303 R380 W524	G24 T69 E158 Y214 E234 S237 W267 Y279 R356 W497	MOAD: Ki=2.3mM PDBbind-CN: -logKd/Ki=2.64,Ki=2.3mM
BS02	CA	A	D477 N479 F511 D660	D450 N452 F484 D633

Gene Ontology

Molecular Function

• GO:0004336 galactosylceramidase activity
• GO:0016798 hydrolase activity, acting on glycosyl bonds

Biological Process

• GO:0006665 sphingolipid metabolic process
• GO:0006683 galactosylceramide catabolic process
• GO:0016042 lipid catabolic process
• GO:0042552 myelination

Cellular Component

• GO:0005739 mitochondrion
• GO:0005764 lysosome

External links

• PDB: RCSB:4ufl, PDBe:4ufl, PDBj:4ufl
• PDBsum: 4ufl
• PubMed: 26029356
• UniProt: P54818|GALC_MOUSE Galactocerebrosidase (Gene Name=Galc)