Details of Protein

General Information of Protein (ID: PRT00321)
Name Ethylmalonyl-CoA decarboxylase (ECHDC1)
Synonyms   Click to Show/Hide Synonyms of This Protein
Enoyl-CoA hydratase domain-containing protein 1; Methylmalonyl-CoA decarboxylase; MMCD; Echdc1
Gene Name Echdc1 Gene ID
52665
UniProt ID
Q9D9V3
Family Lyases (EC 4)
EC Number   EC: 4.1.1.94  (Click to Show/Hide the Complete EC Tree)
Lyases
Carbon-carbon lyase
Carboxy-lyase
EC: 4.1.1.94
  Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein
Sequence
MRRCEVNSKPISEYFGIPCENREMAKCLLTSSLSVRTKLLQTGVSLYNTSHGFHEEEVKK
ILEQFPGGSIDLLKKQNGIGILTLNNPNKMNAFSGVMMLQLLERVIELENWTEGKGLIIH
GAKNTFCSGSDLNAVKALSTPESGVALSMFMQNTLTRFMRLPLISVALVQGWAMGGGAEL
TTACDFRLMTEESVIRFVHKEMGIVPSWGGTSRLVEIIGSRQALKVLSGTLKLDSKEALN
IGLTDEVLQPSDETTALEQAQEWLEKFVSGPPQVIRGLKKSVCSARELYIEEALQNERDV
LETLWGGPANLEAIAKKGKHTK
Function Decarboxylates ethylmalonyl-CoA, a potentially toxic metabolite, to form butyryl-CoA, suggesting it might be involved in metabolite proofreading. Also has methylmalonyl-CoA decarboxylase activity at lower level.
Regulatory Network
Full List of Metabolite(s) Regulated by This Protein
      Lipids and lipid-like molecules
            (S)-Ethylmalonyl-CoA Click to Show/Hide the Full List of Regulating Pair(s):   2 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair (1) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Echdc1
                      Induced Change (S)-Ethylmalonyl-CoA concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that knockout of Echdc1 leads to the increase of (S)-ethylmalonyl-CoA levels compared with control group.
               Regulating Pair (2) Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Overexpression of Echdc1
                      Induced Change (S)-Ethylmalonyl-CoA concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that overexpression of Echdc1 leads to the decrease of (S)-ethylmalonyl-CoA levels compared with control group.
            Methylmalonyl-CoA 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 Overexpression of Echdc1
                      Induced Change Methylmalonyl-CoA concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that overexpression of Echdc1 leads to the decrease of methylmalonyl-CoA levels compared with control group.
            Propionyl-CoA 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 Overexpression of Echdc1
                      Induced Change Propionyl-CoA concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that overexpression of Echdc1 leads to the increase of propionyl-CoA levels compared with control group.
            Succinyl-CoA 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 Knockout of Echdc1
                      Induced Change Succinyl-CoA concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that knockout of Echdc1 leads to the decrease of succinyl-CoA levels compared with control group.
      Organic oxygen compounds
            Acetyl-CoA 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 Overexpression of Echdc1
                      Induced Change Acetyl-CoA concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that overexpression of Echdc1 leads to the increase of acetyl-CoA levels compared with control group.
References
1 The synthesis of branched-chain fatty acids is limited by enzymatic decarboxylation of ethyl- and methylmalonyl-CoA. Biochem J. 2019 Aug 30;476(16):2427-2447.

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