Details of Protein

General Information of Protein (ID: PRT00286)
Name Dihydrodipicolinate synthase-like (DHDPSL)
Synonyms   Click to Show/Hide Synonyms of This Protein
4-hydroxy-2-oxoglutarate aldolase, mitochondrial; DHDPS-like protein; Probable 2-keto-4-hydroxyglutarate aldolase; Probable KHG-aldolase; Protein 569272; HOGA1; C10orf65
Gene Name HOGA1 Gene ID
112817
UniProt ID
Q86XE5
Family Lyases (EC 4)
EC Number   EC: 4.1.3.16  (Click to Show/Hide the Complete EC Tree)
Lyases
Carbon-carbon lyase
Oxo-acid-lyase
EC: 4.1.3.16
  Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein
Sequence
MLGPQVWSSVRQGLSRSLSRNVGVWASGEGKKVDIAGIYPPVTTPFTATAEVDYGKLEEN
LHKLGTFPFRGFVVQGSNGEFPFLTSSERLEVVSRVRQAMPKNRLLLAGSGCESTQATVE
MTVSMAQVGADAAMVVTPCYYRGRMSSAALIHHYTKVADLSPIPVVLYSVPANTGLDLPV
DAVVTLSQHPNIVGMKDSGGDVTRIGLIVHKTRKQDFQVLAGSAGFLMASYALGAVGGVC
ALANVLGAQVCQLERLCCTGQWEDAQKLQHRLIEPNAAVTRRFGIPGLKKIMDWFGYYGG
PCRAPLQELSPAEEEALRMDFTSNGWL
Structure
3S5N ; 3S5O
Function Catalyzes the final step in the metabolic pathway of hydroxyproline.
Regulatory Network
Full List of Metabolite(s) Regulated by This Protein
      Lipids and lipid-like molecules
            2,4-Dihydroxyglutarate 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 (patients: bi-allelic mutations) of HOGA1
                      Induced Change 2,4-Dihydroxyglutarate concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Carbohydrate metabolism disorders [ICD-11: 5C51]
                      Details It is reported that mutation (patients with bi-allelic mutations) of HOGA1 leads to the increase of 2,4-dihydroxyglutarate levels compared with control group.
      Organic acids and derivatives
            4-Hydroxy-L-glutamic 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 (patients: bi-allelic mutations) of HOGA1
                      Induced Change 4-Hydroxy-L-glutamic acid concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Carbohydrate metabolism disorders [ICD-11: 5C51]
                      Details It is reported that mutation (patients with bi-allelic mutations) of HOGA1 leads to the increase of 4-hydroxy-L-glutamic acid levels compared with control group.
            D-4-Hydroxy-2-oxoglutarate 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 (patients: bi-allelic mutations) of HOGA1
                      Induced Change D-4-Hydroxy-2-oxoglutarate concentration: increase (FC = 50)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Carbohydrate metabolism disorders [ICD-11: 5C51]
                      Details It is reported that mutation (patients with bi-allelic mutations) of HOGA1 leads to the increase of D-4-hydroxy-2-oxoglutarate levels compared with control group.
            Oxalic 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 (patients: bi-allelic mutations) of HOGA1
                      Induced Change Oxalic acid concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Carbohydrate metabolism disorders [ICD-11: 5C51]
                      Details It is reported that mutation (patients with bi-allelic mutations) of HOGA1 leads to the increase of oxalic acid levels compared with control group.
References
1 Primary hyperoxaluria type III--a model for studying perturbations in glyoxylate metabolism. J Mol Med (Berl). 2012 Dec;90(12):1497-504.

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