1.2.1.31: L-aminoadipate-semialdehyde dehydrogenase

This is an abbreviated version!
For detailed information about L-aminoadipate-semialdehyde dehydrogenase, go to the full flat file.

Reaction

Synonyms

2-aminoadipate semialdehyde dehydrogenase, 2-aminoadipic 6-semialdehyde dehydrogenase, 2-aminoadipic semialdehyde dehydrogenase, AAR, AASADH, adipic 6-semialdehyde dehydrogenase, aldehyde dehydrogenase 7A1, Aldh7a1, alpha-AAR, alpha-AASA dehydrogenase, alpha-aminoadipate reductase Lys1p, alpha-aminoadipate semialdehyde dehydrogenase, alpha-aminoadipate-semialdehyde dehydrogenase, alpha-aminoadipic acid-delta-semialdehyde dehydrogenase, alpha-aminoadipic semialdehyde dehydrogenase, Alpha-AR, antiquitin, dehydrogenase, aminoadipate semialdehyde, human U26, L-alpha-aminoadipate delta-semialdehyde oxidoreductase, L-alpha-aminoadipate delta-semialdehyde:NAD oxidoreductase, L-alpha-aminoadipate delta-semialdehyde:nicotinamide adenine dinucleotide oxidoreductase, L-aminoadipate-semialdehyde dehydrogenase, Lys1p, Nalpha-Z-L-AASA dehydrogenase, U26

ECTree

     1 Oxidoreductases

         1.2 Acting on the aldehyde or oxo group of donors

             1.2.1 With NAD⁺ or NADP⁺ as acceptor

                1.2.1.31 L-aminoadipate-semialdehyde dehydrogenase

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Results	in table
5	Activating Compound
1347	AA Sequence
1	Application
1	CAS Registry Number
14	Cloned(Commentary)
26	Cofactor
542	Disease/ Diagnostics
1	Expression
6	General Information
4	General Stability
41	Inhibitors
7	Ki Value [mM]
22	KM Value [mM]
5	Localization
14	Metals/Ions
12	Molecular Weight [Da]
23	Natural Substrates/ Products (Substrates)
43	Organism
6	Pathway
131	PDB ID
8	pH Optimum
3	pH Range
1	pH Stability
6	Posttranslational Modification
24	Protein Variants
9	Purification (Commentary)
1	Reaction
5	Reaction Type
41	Reference
82	Source Tissue
22	Specific Activity [micromol/min/mg]
3	Storage Stability
57	Substrates and Products (Substrate)
4	Subunits
42	Synonyms
1	Systematic Name
2	Temperature Optimum [°C]
1	Temperature Range [°C]
8	Temperature Stability [°C]
2	Turnover Number [1/s]

General Information

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General Information on EC 1.2.1.31 - L-aminoadipate-semialdehyde dehydrogenase

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GENERAL INFORMATION

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

malfunction

3 entries

metabolism

2 entries

physiological function

Homo sapiens

P49419

the catalytically active oligomer of ALDH7A1 is assembled on demand in response to cofactor availability

763029

malfunction

Homo sapiens

-

alpha-AASA dehydrogenase deficiency results in the accumulation of pathognomonic alpha-aminoadipic semialdehyde (in cerebrospinal fluid, plasma and urine) and pipecolic acid (cerebrospinal fluid and plasma) in affected patients

712017

malfunction

Homo sapiens

P49419

enzyme deficiency causes pyridoxine-dependent epilepsy

763341

malfunction

Homo sapiens

P49419

mutations in the ALDH7A1 gene can cause the autosomal recessive metabolic disease pyridoxine-dependent epilepsy (PDE), which is characterized by seizure activity within days of birth. Although PDE symptoms can be mitigated with high doses of pyridoxine (vitamin B6) and a lysine-restricted diet, many patients have long-lasting and untreatable cognitive disability. The molecular basis of PDE is thought to be due to a buildup of DELTA1-piperideine-6-carboxylate, the cyclic form of the aldehyde substrate of ALDH7A1

763029

metabolism

Homo sapiens

P49419

human aldehyde dehydrogenase 7A1 (ALDH7A1) catalyzes the final step in lysine catabolism, the NAD+-dependent oxidation of alpha-aminoadipate semialdehyde to alpha-aminoadipate

763029

metabolism

Homo sapiens

P49419

key enzyme in lysine oxidation

763341