Structure of PDB 6o4i Chain D

Receptor sequence
>6o4iD (length=509) Species: 9606 (Homo sapiens) [Search protein sequence]
TLLINQPQYAWLKELGLREENEGVYNGSWGGRGEVITTYCPANNEPIARV
RQASVADYEETVKKAREAWKIWADIPAPKRGEIVRQIGDALREKIQVLGS
LVSLEMGKILVEGVGEVQEYVDICDYAVGLSRMIGGPILPSERSGHALIE
QWNPVGLVGIITAFNFPVAVYGWNNAIAMICGNVCLWKGAPTTSLISVAV
TKIIAKVLEDNKLPGAICSLTCGGADIGTAMAKDERVNLLSFTGSTQVGK
QVGLMVQERFGRSLLELGGNNAIIAFEDADLSLVVPSALFAAVGTAGQRC
TTARRLFIHESIHDEVVNRLKKAYAQIRVGNPWDPNVLYGPLHTKQAVSM
FLGAVEEAKKEGGTVVYGGKVMDRPGNYVEPTIVTGLGHDASIAHTDTFA
PILYVFKFKNEEEVFAWNNEVKQGLSSSIFTKDLGRIFRWLGPKGSDCGI
VNVNIPTSGAEIGGAFGGEKHTGGGRESGSDAWKQYMRRSTCTINYSKDL
PLAQGIKFQ
3D structure
PDB6o4i Structural analysis of pathogenic mutations targeting Glu427 of ALDH7A1, the hot spot residue of pyridoxine-dependent epilepsy.
ChainD
Resolution1.75 Å
3D
structure
Catalytic site residues are labeled in the structure
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Enzymatic activity
Catalytic site (original residue number in PDB) N167 E268 C302 E479
Catalytic site (residue number reindexed from 1) N165 E266 C300 E477
Enzyme Commision number 1.2.1.3: aldehyde dehydrogenase (NAD(+)).
1.2.1.31: L-aminoadipate-semialdehyde dehydrogenase.
1.2.1.8: betaine-aldehyde dehydrogenase.
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 UN1 D E121 F168 W175 R301 C302 T303 S460 G461 A462 F468 E119 F166 W173 R299 C300 T301 S458 G459 A460 F466
Gene Ontology
Molecular Function
GO:0004029 aldehyde dehydrogenase (NAD+) activity
GO:0004043 L-aminoadipate-semialdehyde dehydrogenase activity
GO:0005515 protein binding
GO:0008802 betaine-aldehyde dehydrogenase (NAD+) activity
GO:0016491 oxidoreductase activity
GO:0016620 oxidoreductase activity, acting on the aldehyde or oxo group of donors, NAD or NADP as acceptor
GO:0042802 identical protein binding
Biological Process
GO:0006081 cellular aldehyde metabolic process
GO:0007605 sensory perception of sound
GO:0019285 glycine betaine biosynthetic process from choline
GO:0042426 choline catabolic process
Cellular Component
GO:0005634 nucleus
GO:0005737 cytoplasm
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0005829 cytosol
GO:0070062 extracellular exosome

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Molecular Function
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Biological Process
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Cellular Component
External links
PDB RCSB:6o4i, PDBe:6o4i, PDBj:6o4i
PDBsum6o4i
PubMed31652343
UniProtP49419|AL7A1_HUMAN Alpha-aminoadipic semialdehyde dehydrogenase (Gene Name=ALDH7A1)

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