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Protein Page:
ALDH7A1 (human)
p Phosphorylation
a Acetylation
m Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
u Ubiquitination
s Sumoylation
n Neddylation
gl O-GlcNAc
ga O-GalNAc
h Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage

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: EC 1.2.1.8; Amino Acid Metabolism - tryptophan; Oxidoreductase; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - histidine; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - lysine degradation; Other Amino Acids Metabolism - beta-alanine; EC 1.2.1.3; EC 1.2.1.31; Secondary Metabolites Metabolism - limonene and pinene degradation; Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - glycerolipid; Lipid Metabolism - fatty acid; Carbohydrate Metabolism - ascorbate and aldarate; Carbohydrate Metabolism - pyruvate; Mitochondrial; Carbohydrate Metabolism - glycolysis and gluconeogenesis
Cellular Component: mitochondrial matrix; nucleus; cytosol
Molecular Function: L-aminoadipate-semialdehyde dehydrogenase activity; protein binding; aldehyde dehydrogenase (NAD) activity; betaine-aldehyde dehydrogenase activity
Biological Process: sensory perception of sound; glycine betaine biosynthetic process from choline; lysine catabolic process; aldehyde metabolic process
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.


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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


 SS 

SS: 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.


 MS 

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


       human

► Hide Isoforms
 
0 15 Y69-p RGEVITTyCPANNEP
0 13 S84-p IARVRQAsVADYEET
0 4 A86 RVRQAsVADYEETVk
0 1 A86 RVRQAsVADYEETVk
0 17 K93-a ADYEETVkkAREAWK
0 4 K94-a DYEETVkkAREAWKI
0 1 K94 DYEETVkKAREAWKI
0 1 E97 ETVkkAREAWKIWAD
0 2 K109-u WADIPAPkRGEIVRQ
0 1 Q116 kRGEIVRQIGDALRE
0 1 K124 IGDALREKIQVLGsL
0 1 S130-p EKIQVLGsLVsLEMG
0 1 S133-p QVLGsLVsLEMGKIL
0 1 S171-p IGGPILPsERSGHAL
0 1 S379-p LHTKQAVsMFLGAVE
0 9 K389-a LGAVEEAkKEGGTVV
0 1 K439-a ILYVFKFkNEEEVFA
0 5 K452-u FAWNNEVkQGLSSSI
0 2 K462 LSSSIFTKDLGRIFR
0 1 K500 GGAFGGEKHTGGGRE
0 1 K514 ESGSDAWKQYMRRST
0 1 K528-u TCTINYSkDLPLAQG
0 1 K528 TCTINYSKDLPLAQG
0 1 K537-u LPLAQGIkFQ_____
  ALDH7A1 iso4  
Y96 RGEVITTYCPANNEP
S111 IARVRQASVADYEET
A113 RVRQASVADYEETVK
A113 RVRQASVADYEETVK
K120 ADYEETVKKAREAWK
K121 DYEETVKKAREAWKI
K121 DYEETVKKAREAWKI
E124 ETVKKAREAWKIWAD
K136 WADIPAPKRGEIVRQ
Q143 KRGEIVRQIGDALRE
K151 IGDALREKIQVLGSL
S157 EKIQVLGSLVSLEMG
S160 QVLGSLVSLEMGKIL
S198 IGGPILPSERSGHAL
- gap
- gap
K402 ILYVFKFKNEEEVFA
K415 FAWNNEVKQGLSSSI
K425 LSSSIFTKDLGRIFR
K463 GGAFGGEKHTGGGRE
K477 ESGSDAWKQYMRRST
K491 TCTINYSKDLPLAQG
K491 TCTINYSKDLPLAQG
K500 LPLAQGIKFQ_____
  mouse

 
Y69-p RGEVITTyCPANNEP
S84 IARVRQASLkDYEET
K86-a RVRQASLkDYEETIG
K86-u RVRQASLkDYEETIG
G93 kDYEETIGkAKkAWN
K94-a DYEETIGkAKkAWNI
K94-u DYEETIGkAKkAWNI
K97-u ETIGkAKkAWNIWAD
K109-u WADIPAPkRGEIVRk
K116-u kRGEIVRkIGDAFRE
K124-u IGDAFREkIQLLGRL
R130 EkIQLLGRLVSLEMG
S133 QLLGRLVSLEMGKIL
S171 IGGPTLPSERPGHAL
S379 LHTKQAVSMFVRAVE
K389-a VRAVEEAkKQGGTVV
Q439 ILYVFKFQDEEEVFE
K452-u FEWNNEVkQGLSSSI
K462-u LSSSIFTkDLGRIFR
K500-u GGAFGGEkHTGGGRE
K514-u ESGSDAWkQYMRRST
T528 TCTINYSTSLPLAQG
T528-p TCTINYStSLPLAQG
K537 LPLAQGIKFQ_____
  rat

 
Y69-p RGEVITTyCPANNEP
S84 IARVRQASMKDYEET
K86 RVRQASMKDYEETIG
K86 RVRQASMKDYEETIG
G93 KDYEETIGKAKKAWN
K94 DYEETIGKAKKAWNI
K94 DYEETIGKAKKAWNI
K97 ETIGKAKKAWNIWAD
K109 WADIPAPKRGEIVRK
K116 KRGEIVRKIGDALRE
K124 IGDALREKIQLLGRL
R130 EKIQLLGRLVSLEMG
S133 QLLGRLVSLEMGKIL
S171 IGGPTLPSERPGHAL
S379 LHTKQAVSMFVQAVE
K389 VQAVEEAKKEGGTVV
K439 ILYVFKFKNEEEVFE
K452 FEWNNEVKQGLSSSI
K462 LSSSIFTKDLGRIFR
K500 GGAFGGEKHTGGGRE
K514 ESGSDAWKQYMRRST
T528 TCTINYSTALPLAQG
T528 TCTINYSTALPLAQG
K537 LPLAQGIKFQ_____
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