Details of Protein

General Information of Protein (ID: PRT00269)
Name Formyltetrahydrofolate dehydrogenase (ALDH1L2)
Synonyms   Click to Show/Hide Synonyms of This Protein
Mitochondrial 10-FTHFDH; mtFDH; Aldehyde dehydrogenase family 1 member L2; ALDH1L2
Gene Name ALDH1L2 Gene ID
160428
UniProt ID
Q3SY69
Family Oxidoreductases (EC 1)
EC Number   EC: 1.5.1.6  (Click to Show/Hide the Complete EC Tree)
Oxidoreductase
CH-NH donor oxidoreductase
NAD/NADP acceptor oxidoreductase
EC: 1.5.1.6
  Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein
Sequence
MLRRGSQALRRFSTGRVYFKNKLKLALIGQSLFGQEVYSHLRKEGHRVVGVFTVPDKDGK
ADPLALAAEKDGTPVFKLPKWRVKGKTIKEVAEAYRSVGAELNVLPFCTQFIPMDIIDSP
KHGSIIYHPSILPRHRGASAINWTLIMGDKKAGFSVFWADDGLDTGPILLQRSCDVEPND
TVDALYNRFLFPEGIKAMVEAVQLIADGKAPRIPQPEEGATYEGIQKKENAEISWDQSAE
VLHNWIRGHDKVPGAWTEINGQMVTFYGSTLLNSSVPPGEPLEIKGAKKPGLVTKNGLVL
FGNDGKALTVRNLQFEDGKMIPASQYFSTGETSVVELTAEEVKVAETIKVIWAGILSNVP
IIEDSTDFFKSGASSMDVARLVEEIRQKCGGLQLQNEDVYMATKFEGFIQKVVRKLRGED
QEVELVVDYISKEVNEIMVKMPYQCFINGQFTDADDGKTYDTINPTDGSTICKVSYASLA
DVDKAVAAAKDAFENGEWGRMNARERGRLMYRLADLLEENQEELATIEALDSGAVYTLAL
KTHIGMSVQTFRYFAGWCDKIQGSTIPINQARPNRNLTFTKKEPLGVCAIIIPWNYPLMM
LAWKSAACLAAGNTLVLKPAQVTPLTALKFAELSVKAGFPKGVINIIPGSGGIAGQRLSE
HPDIRKLGFTGSTPIGKQIMKSCAVSNLKKVSLELGGKSPLIIFNDCELDKAVRMGMGAV
FFNKGENCIAAGRLFVEESIHDEFVTRVVEEIKKMKIGDPLDRSTDHGPQNHKAHLEKLL
QYCETGVKEGATLVYGGRQVQRPGFFMEPTVFTDVEDYMYLAKEESFGPIMVISKFQNGD
IDGVLQRANSTEYGLASGVFTRDINKAMYVSEKLEAGTVFINTYNKTDVAAPFGGVKQSG
FGKDLGEEALNEYLKTKTVTLEY
Regulatory Network
Full List of Metabolite(s) Regulated by This Protein
      Nucleosides, nucleotides, and analogues
            ATP Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change ATP concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the decrease of ATP levels compared with control group.
      Benzenoids
            Norepinephrine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Norepinephrine concentration: increase (FC = 4)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of norepinephrine levels compared with control group.
      Lipid-related molecules
            Linoelaidylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Linoelaidylcarnitine concentration: increase (FC = 7.14)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of linoelaidylcarnitine levels compared with control group.
            Margaroylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Margaroylcarnitine concentration: increase (FC = 0.72)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of margaroylcarnitine levels compared with control group.
      Lipids and lipid-like molecules
            3-Hydroxybutyrylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change 3-Hydroxybutyrylcarnitine concentration: increase (FC = 2.86)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of 3-hydroxybutyrylcarnitine levels compared with control group.
            4-Trimethylammoniobutanoic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change 4-Trimethylammoniobutanoic acid concentration: increase (FC = 4.76)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of 4-trimethylammoniobutanoic acid levels compared with control group.
            5-Dodecenoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change 5-Dodecenoylcarnitine concentration: increase (FC = 2.94)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of 5-dodecenoylcarnitine levels compared with control group.
            9-Hexadecenoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change 9-Hexadecenoylcarnitine concentration: increase (FC = 7.69)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of 9-hexadecenoylcarnitine levels compared with control group.
            Arachidonoyl carnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Arachidonoyl carnitine concentration: increase (FC = 2.27)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of arachidonoyl carnitine levels compared with control group.
            Butyrylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Butyrylcarnitine concentration: increase (FC = 3.85)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of butyrylcarnitine levels compared with control group.
            cis-4-Decenoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change cis-4-Decenoylcarnitine concentration: increase (FC = 5.26)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of cis-4-decenoylcarnitine levels compared with control group.
            Decanoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Decanoylcarnitine concentration: increase (FC = 7.14)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of decanoylcarnitine levels compared with control group.
            Docosapentaenoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Docosapentaenoylcarnitine concentration: increase (FC = 1.56)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of docosapentaenoylcarnitine levels compared with control group.
            Dodecanoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Dodecanoylcarnitine concentration: increase (FC = 9.09)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of dodecanoylcarnitine levels compared with control group.
            Hexanoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Hexanoylcarnitine concentration: increase (FC = 5.56)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of hexanoylcarnitine levels compared with control group.
            L-Acetylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change L-Acetylcarnitine concentration: increase (FC = 7.14)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of L-acetylcarnitine levels compared with control group.
            L-Octanoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change L-Octanoylcarnitine concentration: increase (FC = 5.88)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of L-octanoylcarnitine levels compared with control group.
            Myristoleoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Myristoleoylcarnitine concentration: increase (FC = 8.33)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of myristoleoylcarnitine levels compared with control group.
            Oleoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Oleoylcarnitine concentration: increase (FC = 4.76)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of oleoylcarnitine levels compared with control group.
            Pentadecanoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Pentadecanoylcarnitine concentration: increase (FC = 7.69)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of pentadecanoylcarnitine levels compared with control group.
            Propionylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Propionylcarnitine concentration: increase (FC = 4)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of propionylcarnitine levels compared with control group.
            Stearoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Stearoylcarnitine concentration: increase (FC = 1.92)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of stearoylcarnitine levels compared with control group.
            Tetradecanoylcarnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Tetradecanoylcarnitine concentration: increase (FC = 6.25)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of tetradecanoylcarnitine levels compared with control group.
      Organic acids and derivatives
            cis-Aconitic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change cis-Aconitic acid concentration: increase (FC = 3.57)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of cis-aconitic acid levels compared with control group.
            Citric acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Citric acid concentration: increase (FC = 2.70)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of citric acid levels compared with control group.
            Malic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Malic acid concentration: increase (FC = 2.78)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of malic acid levels compared with control group.
            Oxoglutaric acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Oxoglutaric acid concentration: increase (FC = 2.78)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of oxoglutaric acid levels compared with control group.
            Succinic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Succinic acid concentration: increase (FC = 3.33)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of succinic acid levels compared with control group.
      Organic nitrogen compounds
            Carnitine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Carnitine concentration: increase (FC = 3.23)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of carnitine levels compared with control group.
            Dihomo-gamma-linolenoylcholine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Dihomo-gamma-linolenoylcholine concentration: increase (FC = 4)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of dihomo-gamma-linolenoylcholine levels compared with control group.
      Organic oxygen compounds
            Glycerol Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change Glycerol concentration: increase (FC = 2.63)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of glycerol levels compared with control group.
Full List of Metabolite(s) Regulating This Protein
      Organic acids and derivatives
            Glutamine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [2]
                      Introduced Variation Glutamine absence (16 hours)
                      Induced Change ALDH1L2 protein abundance levels: increase (FC = 1.82)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Hepatocellular carcinoma [ICD-11: 2C12]
                      Details It is reported that glutamine absence causes the increase of ALDH1L2 protein abundance compared with control group.
References
1 Deleterious mutations in ALDH1L2 suggest a novel cause for neuro-ichthyotic syndrome. NPJ Genom Med. 2019 Jul 23;4:17.
2 Quantitative proteomics analysis reveals glutamine deprivation activates fatty acid -oxidation pathway in HepG2 cells. Amino Acids. 2016 May;48(5):1297-307.

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