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Protein Page:
ALDH7A1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitylation
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
ALDH7A1 Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism. Defects in ALDH7A1 are the cause of pyridoxine-dependent epilepsy (PDE). PDE is characterized by a combination of various seizure types. It usually occurs in the first hours of life and is unresponsive to standard anticonvulsants, responding only to immediate administration of pyridoxine hydrochloride. Belongs to the aldehyde dehydrogenase family. 3 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - lysine degradation; Mitochondrial; Lipid Metabolism - glycerolipid; EC 1.2.1.3; Carbohydrate Metabolism - glycolysis and gluconeogenesis; EC 1.2.1.8; Amino Acid Metabolism - histidine; Lipid Metabolism - fatty acid; Carbohydrate Metabolism - ascorbate and aldarate; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - arginine and proline; Other Amino Acids Metabolism - beta-alanine; EC 1.2.1.31; Carbohydrate Metabolism - butanoate; Carbohydrate Metabolism - propanoate; Secondary Metabolites Metabolism - limonene and pinene degradation; Carbohydrate Metabolism - pyruvate; Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation
Chromosomal Location of Human Ortholog: 5q31
Cellular Component: cytoplasm; mitochondrial matrix; mitochondrion
Molecular Function: aldehyde dehydrogenase (NAD) activity; betaine-aldehyde dehydrogenase activity; L-aminoadipate-semialdehyde dehydrogenase activity; protein binding
Biological Process: aldehyde metabolic process; choline catabolic process; lysine catabolic process; sensory perception of sound
Disease: Epilepsy, Pyridoxine-dependent
Reference #:  P49419 (UniProtKB)
Alt. Names/Synonyms: 26g turgor protein homolog; AL7A1; aldehyde dehydrogenase 7 family, member A1; Aldehyde dehydrogenase family 7 member A1; ALDH7A1; Alpha-AASA dehydrogenase; Alpha-aminoadipic semialdehyde dehydrogenase; antiquitin 1; Antiquitin-1; ATQ1; Betaine aldehyde dehydrogenase; Delta1-piperideine-6-carboxylate dehydrogenase; delta1-piperideine-6-carboxylate dehydrogenease; EPD; FLJ11738; FLJ92814; P6c dehydrogenase; PDE
Gene Symbols: ALDH7A1
Molecular weight: 58,487 Da
Basal Isoelectric point: 8.21  Predict pI for various phosphorylation states
Select Structure to View Below

ALDH7A1

Protein Structure Not Found.
Download PyMol Script
Download ChimeraX Script


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment



 LTP 

LTP: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 HTP 

HTP: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

► Hide Isoforms
 
0 1 - gap
0 1 - gap
0 1 - gap
0 1 Y39‑p LLINQPQyAWLKELG
0 20 Y69‑p RGEVITTyCPANNEP
0 16 S84‑p IARVRQAsVADyEET
0 6 A86 RVRQAsVADyEETVk
0 1 A86 RVRQAsVADyEETVk
0 2 A86 RVRQAsVADyEETVk
0 1 Y88‑p RQAsVADyEETVkkA
0 17 K93‑ac ADyEETVkkAREAWK
0 1 K93‑sc ADyEETVkkAREAWK
0 6 K94‑ac DyEETVkkAREAWKI
0 1 K94 DyEETVkKAREAWKI
0 2 K94 DyEETVkKAREAWKI
0 1 E97 ETVkkAREAWKIWAD
0 1 E97 ETVkkAREAWKIWAD
0 1 E97 ETVkkAREAWKIWAD
0 1 K109 WADIPAPKRGEIVRQ
0 2 K109‑ub WADIPAPkRGEIVRQ
0 1 Q116 kRGEIVRQIGDALRE
0 1 K124 IGDALREKIQVLGsL
0 1 K124 IGDALREKIQVLGsL
0 1 K124 IGDALREKIQVLGsL
0 1 S130‑p EKIQVLGsLVsLEMG
0 1 S133‑p QVLGsLVsLEMGKIL
0 1 S171‑p IGGPILPsERSGHAL
0 1 K351 DEVVNRLKKAYAQIR
0 1 K375 LYGPLHTKQAVsMFL
0 1 K375 LYGPLHTKQAVsMFL
0 2 S379‑p LHTKQAVsMFLGAVE
0 11 K389‑ac LGAVEEAkKEGGTVV
0 1 K389‑sc LGAVEEAkKEGGTVV
0 3 K390 GAVEEAkKEGGTVVY
0 1 K390 GAVEEAkKEGGTVVY
0 1 K400 GTVVYGGKVMDRPGN
0 1 T426 HDASIAHTETFAPIL
0 3 K439‑ac ILYVFKFkNEEEVFA
0 1 K439‑sc ILYVFKFkNEEEVFA
0 2 K452 FAWNNEVKQGLSsSI
0 5 K452‑ub FAWNNEVkQGLSsSI
0 1 K452 FAWNNEVKQGLSsSI
0 1 S457‑p EVkQGLSsSIFTkDL
0 2 K462 LSsSIFTKDLGRIFR
0 2 K462 LSsSIFTKDLGRIFR
0 1 K462‑sc LSsSIFTkDLGRIFR
0 1 K474 IFRWLGPKGSDCGIV
0 1 K500 GGAFGGEKHTGGGRE
0 1 K500 GGAFGGEKHTGGGRE
0 1 K514 ESGSDAWKQYMRRST
0 1 K514 ESGSDAWKQYMRRST
0 1 K514 ESGSDAWKQYMRRST
0 1 T521 KQYMRRSTCTINYSk
0 1 K528‑ub TCTINYSkDLPLAQG
0 1 K528‑sc TCTINYSkDLPLAQG
0 1 K528 TCTINYSKDLPLAQG
0 1 K528‑ac TCTINYSkDLPLAQG
0 1 K537‑ub LPLAQGIkFQ_____
  ALDH7A1 iso2  
- gap
- gap
- gap
Y11 LLINQPQYAWLKELG
Y41 RGEVITTYCPANNEP
S56 IARVRQASVADYEET
A58 RVRQASVADYEETVK
A58 RVRQASVADYEETVK
A58 RVRQASVADYEETVK
Y60 RQASVADYEETVKKA
K65 ADYEETVKKAREAWK
K65 ADYEETVKKAREAWK
K66 DYEETVKKAREAWKI
K66 DYEETVKKAREAWKI
K66 DYEETVKKAREAWKI
E69 ETVKKAREAWKIWAD
E69 ETVKKAREAWKIWAD
E69 ETVKKAREAWKIWAD
K81 WADIPAPKRGEIVRQ
K81 WADIPAPKRGEIVRQ
Q88 KRGEIVRQIGDALRE
K96 IGDALREKIQVLGSL
K96 IGDALREKIQVLGSL
K96 IGDALREKIQVLGSL
S102 EKIQVLGSLVSLEMG
S105 QVLGSLVSLEMGKIL
S143 IGGPILPSERSGHAL
K323 DEVVNRLKKAYAQIR
K347 LYGPLHTKQAVSMFL
K347 LYGPLHTKQAVSMFL
S351 LHTKQAVSMFLGAVE
K361 LGAVEEAKKEGGTVV
K361 LGAVEEAKKEGGTVV
K362 GAVEEAKKEGGTVVY
K362 GAVEEAKKEGGTVVY
K372 GTVVYGGKVMDRPGN
T398 HDASIAHTETFAPIL
K411 ILYVFKFKNEEEVFA
K411 ILYVFKFKNEEEVFA
K424 FAWNNEVKQGLSSSI
K424 FAWNNEVKQGLSSSI
K424 FAWNNEVKQGLSSSI
S429 EVKQGLSSSIFTKDL
K434 LSSSIFTKDLGRIFR
K434 LSSSIFTKDLGRIFR
K434 LSSSIFTKDLGRIFR
K446 IFRWLGPKGSDCGIV
K472 GGAFGGEKHTGGGRE
K472 GGAFGGEKHTGGGRE
K486 ESGSDAWKQYMRRST
K486 ESGSDAWKQYMRRST
K486 ESGSDAWKQYMRRST
T493 KQYMRRSTCTINYSK
K500 TCTINYSKDLPLAQG
K500 TCTINYSKDLPLAQG
K500 TCTINYSKDLPLAQG
K500 TCTINYSKDLPLAQG
K509 LPLAQGIKFQ_____
  ALDH7A1 iso4  
S13‑p RGAGLYFsSsQGLGL
S15‑p AGLYFsSsQGLGLIP
S23‑p QGLGLIPsPGLSMWR
Y66 LLINQPQYAWLKELG
Y96 RGEVITTYCPANNEP
S111 IARVRQASVADYEET
A113 RVRQASVADYEETVK
A113 RVRQASVADYEETVK
A113 RVRQASVADYEETVK
Y115 RQASVADYEETVKKA
K120 ADYEETVKKAREAWK
K120 ADYEETVKKAREAWK
K121 DYEETVKKAREAWKI
K121 DYEETVKKAREAWKI
K121 DYEETVKKAREAWKI
E124 ETVKKAREAWKIWAD
E124 ETVKKAREAWKIWAD
E124 ETVKKAREAWKIWAD
K136 WADIPAPKRGEIVRQ
K136 WADIPAPKRGEIVRQ
Q143 KRGEIVRQIGDALRE
K151 IGDALREKIQVLGSL
K151 IGDALREKIQVLGSL
K151 IGDALREKIQVLGSL
S157 EKIQVLGSLVSLEMG
S160 QVLGSLVSLEMGKIL
S198 IGGPILPSERSGHAL
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
- gap
T389 HDASIAHTETFAPIL
K402 ILYVFKFKNEEEVFA
K402 ILYVFKFKNEEEVFA
K415 FAWNNEVKQGLSSSI
K415 FAWNNEVKQGLSSSI
K415 FAWNNEVKQGLSSSI
S420 EVKQGLSSSIFTKDL
K425 LSSSIFTKDLGRIFR
K425 LSSSIFTKDLGRIFR
K425 LSSSIFTKDLGRIFR
K437 IFRWLGPKGSDCGIV
K463 GGAFGGEKHTGGGRE
K463 GGAFGGEKHTGGGRE
K477 ESGSDAWKQYMRRST
K477 ESGSDAWKQYMRRST
K477 ESGSDAWKQYMRRST
T484 KQYMRRSTCTINYSK
K491 TCTINYSKDLPLAQG
K491 TCTINYSKDLPLAQG
K491 TCTINYSKDLPLAQG
K491 TCTINYSKDLPLAQG
K500 LPLAQGIKFQ_____
  mouse

 
- gap
- gap
- gap
Y39 LLIHHPQYAWLQDLG
Y69‑p RGEVITTyCPANNEP
S84 IARVRQASLkDYEET
K86‑ac RVRQASLkDYEETIG
K86‑ub RVRQASLkDYEETIG
K86‑sc RVRQASLkDYEETIG
Y88 RQASLkDYEETIGkA
G93 kDYEETIGkAKkAWN
G93 kDYEETIGkAKkAWN
K94‑ac DYEETIGkAKkAWNI
K94‑ub DYEETIGkAKkAWNI
K94‑sc DYEETIGkAKkAWNI
K97 ETIGkAKKAWNIWAD
K97‑ub ETIGkAKkAWNIWAD
K97‑sc ETIGkAKkAWNIWAD
K109 WADIPAPKRGEIVRk
K109‑ub WADIPAPkRGEIVRk
K116‑ub kRGEIVRkIGDAFRE
K124 IGDAFREKIQLLGRL
K124‑ub IGDAFREkIQLLGRL
K124‑sc IGDAFREkIQLLGRL
R130 EkIQLLGRLVSLEMG
S133 QLLGRLVSLEMGKIL
S171 IGGPTLPSERPGHAL
R351 NEVVDRLRSAYSQIR
K375 LYGPLHTKQAVSMFV
K375‑sc LYGPLHTkQAVSMFV
S379 LHTkQAVSMFVRAVE
K389‑ac VRAVEEAkkQGGTVV
K389‑sc VRAVEEAkkQGGTVV
K390‑ac RAVEEAkkQGGTVVY
K390‑sc RAVEEAkkQGGTVVY
K400 GTVVYGGKVMDHPGN
Q426 HDAPIVHQETFAPIL
Q439 ILYVFKFQDEEEVFE
Q439 ILYVFKFQDEEEVFE
K452‑ac FEWNNEVkQGLSSSI
K452‑ub FEWNNEVkQGLSSSI
K452‑sc FEWNNEVkQGLSSSI
S457 EVkQGLSSSIFTkDL
K462‑ac LSSSIFTkDLGRIFR
K462‑ub LSSSIFTkDLGRIFR
K462‑sc LSSSIFTkDLGRIFR
K474‑ac IFRWLGPkGSDCGIV
K500‑ac GGAFGGEkHTGGGRE
K500‑ub GGAFGGEkHTGGGRE
K514‑ac ESGSDAWkQYMRRSt
K514‑ub ESGSDAWkQYMRRSt
K514‑sc ESGSDAWkQYMRRSt
T521‑p kQYMRRStCTINYSt
T528 tCTINYSTSLPLAQG
T528 tCTINYSTSLPLAQG
T528‑p tCTINYStSLPLAQG
T528 tCTINYSTSLPLAQG
K537 LPLAQGIKFQ_____
  rat

 
- gap
- gap
- gap
Y39 LLIHHPQYAWLQDLG
Y69‑p RGEVITTyCPANNEP
S84 IARVRQASMkDYEET
K86‑ac RVRQASMkDYEETIG
K86 RVRQASMKDYEETIG
K86 RVRQASMKDYEETIG
Y88 RQASMkDYEETIGkA
G93 kDYEETIGkAKkAWN
G93 kDYEETIGkAKkAWN
K94‑ac DYEETIGkAKkAWNI
K94 DYEETIGKAKkAWNI
K94 DYEETIGKAKkAWNI
K97‑ac ETIGkAKkAWNIWAD
K97 ETIGkAKKAWNIWAD
K97 ETIGkAKKAWNIWAD
K109‑ac WADIPAPkRGEIVRK
K109 WADIPAPKRGEIVRK
K116 kRGEIVRKIGDALRE
K124‑ac IGDALREkIQLLGRL
K124 IGDALREKIQLLGRL
K124 IGDALREKIQLLGRL
R130 EkIQLLGRLVSLEMG
S133 QLLGRLVSLEMGKIL
S171 IGGPTLPSERPGHAL
K351‑ac DEVVDRLkNAYSQIR
K375‑ac LYGPLHTkQAVSMFV
K375 LYGPLHTKQAVSMFV
S379 LHTkQAVSMFVQAVE
K389‑ac VQAVEEAkkEGGTVV
K389 VQAVEEAKkEGGTVV
K390‑ac QAVEEAkkEGGTVVY
K390 QAVEEAkKEGGTVVY
K400‑ac GTVVYGGkVMDHPGN
K426‑ac HDAPIVHkETFAPIL
K439‑ac ILYVFKFkNEEEVFE
K439 ILYVFKFKNEEEVFE
K452‑ac FEWNNEVkQGLSSSI
K452 FEWNNEVKQGLSSSI
K452 FEWNNEVKQGLSSSI
S457 EVkQGLSSSIFTkDL
K462‑ac LSSSIFTkDLGRIFR
K462 LSSSIFTKDLGRIFR
K462 LSSSIFTKDLGRIFR
K474 IFRWLGPKGSDCGIV
K500 GGAFGGEKHTGGGRE
K500 GGAFGGEKHTGGGRE
K514 ESGSDAWKQYMRRST
K514 ESGSDAWKQYMRRST
K514 ESGSDAWKQYMRRST
T521 KQYMRRSTCTINYST
T528 TCTINYSTALPLAQG
T528 TCTINYSTALPLAQG
T528 TCTINYSTALPLAQG
T528 TCTINYSTALPLAQG
K537 LPLAQGIKFQ_____
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