Aldehyde dehydrogenases

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The aldehyde dehydrogenase (ALDH) comprise a plurality of groups of enzymes , in the metabolism of many living beings aldehydes oxidize. ALDH are oxidoreductases . To date, nineteen ALDH - genes have been described in people who are largely specific for different substrates are. The ALDH sometimes show clear differences in their amino acid sequences , but can be classified into several groups (ALDH-1, -2 etc.). The individual ALDH are mainly found in the liver and are either cytosolic or mitochondrial .

List of ALDH in humans

The human aldehyde dehydrogenases are usually indispensable in the metabolism. Mutations and corresponding hereditary rare deficiency diseases are known of several enzymes .

Genes (human) Enzyme name Substrate EC number Remarks
ALDH1A1 Retinal dehydrogenase 1 Retinal EC  1.2.1.36
ALDH1A2 Retinal dehydrogenase 2 Retinal EC  1.2.1.36
ALDH1A3 Aldehyde dehydrogenase 1A3 Retinal EC  1.2.1.5
ALDH1L1 10-formyl tetrahydrofolate dehydrogenase 10-formyl tetrahydrofolate EC  1.5.1.6
ALDH1L2 10-formyl tetrahydrofolate dehydrogenase 10-formyl tetrahydrofolate EC  1.5.1.6
ALDH2 Aldehyde dehydrogenase 2 broad spectrum EC  1.2.1.3 Mitochondria ; Ethanol degradation ( acetaldehyde )
ALDH3A1 Aldehyde dehydrogenase 3A1 aromatic aldehydes EC  1.2.1.5
ALDH3A2 Fatty aldehyde dehydrogenase long-chain fatty aldehydes (C 6 to C 24 ), saturated or unsaturated EC  1.2.1.48 Microsomes ; Sjogren Larsson Syndrome
ALDH3B1 Aldehyde dehydrogenase 3B1 medium and long chain saturated and unsaturated aldehydes EC  1.2.1.5 Kidneys , lungs
ALDH3B2 Aldehyde dehydrogenase 3B2 ? EC  1.2.1.5
ALDH4A1 P5C dehydrogenase (S) -1- pyrroline-5-carboxylate , semialdehydes EC  1.5.1.12 Mitochondria; Proline degradation ; Type 2 hyperprolinaemia
ALDH5A1 Succinate semialdehyde dehydrogenase (SSADH) Succinate semialdehyde EC  1.2.1.24 multiple tissue types; GABA degradation; SSADH deficiency
ALDH6A1 Methylmalonate semialdehyde dehydrogenase (MMSDH) Methylmalonate semialdehyde EC  1.2.1.18 , EC  1.2.1.27 Mitochondria; Valine / pyrimidine degradation ; MMSDH deficiency
ALDH7A1 alpha-AASA dehydrogenase L-2-aminoadipate-6-semialdehyde, betaine aldehyde EC  1.2.1.31 , EC  1.2.1.8 Active in the fetus ; Betaine biosynthesis; pyridoxine- dependent epilepsy
ALDH8A1 Aldehyde dehydrogenase 8A1 9-cis-retinal, 13-cis-retinal, benzaldehyde , decanal EC  1.2.1.-
ALDH9A1 Trimethylaminobutyraldehyde dehydrogenase 4-trimethylaminobutyraldehyde; broad spectrum EC  1.2.1.47 Liver, muscles, kidneys; Betaine and carnitine biosynthesis
ALDH16A1 inactive?

Alcohol breakdown

In the metabolism of alcohol , the aldehyde dehydrogenases, especially ALDH2, play an essential role in the detoxification of metabolic products of ethanol . Ethanol is converted into the toxic acetaldehyde by alcohol dehydrogenases (ADH) within the metabolism . ALDH oxidizes the acetaldehyde to the non-toxic acetate . A genetic polymorphism of ALDH-2 occurs in parts of the Asian population , which leads to a rapid surge of acetaldehyde after consumption of even small amounts of ethanol ( flush syndrome ).

These clinical symptoms are also used in the therapy of alcohol addicts. The administration of disulfiram (Antabuse) leads to an inhibition of ALDH-2, which leads to alcohol intolerance.

Individual evidence

  1. ^ Hyperprolinemia, Type II.  In: Online Mendelian Inheritance in Man . (English).
  2. ^ Orphanet: 4-Hydroxybutyraziduria .
  3. ALDH6A1.  In: Online Mendelian Inheritance in Man . (English).
  4. Epilepsy, pyridoxine-dependent.  In: Online Mendelian Inheritance in Man . (English).
  5. Alcohol sensitivity.  In: Online Mendelian Inheritance in Man . (English).