HOGA1

HOGA1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	3S5N, 3S5O
Identifiers
Aliases	HOGA1, C10orf65, DHDPS2, DHDPSL, HP3, NPL2, 4-hydroxy-2-oxoglutarate aldolase 1
External IDs	OMIM: 613597; MGI: 1914682; HomoloGene: 12130; GeneCards: HOGA1; OMA:HOGA1 - orthologs
Gene location (Human)
Chr.	Chromosome 10 (human)
End	97,612,802 bp
Gene location (Mouse)
Chr.	Chromosome 19 (mouse)
End	42,059,392 bp
RNA expression pattern
	Top expressed in
	right lobe of liver; ; renal medulla; ; human kidney; ; apex of heart; ; left ventricle; ; right adrenal cortex; ; body of pancreas; ; left adrenal gland; ; C1 segment; ; right auricle of heart;
	Top expressed in
	right kidney; ; proximal tubule; ; liver; ; left lobe of liver; ; human kidney; ; yolk sac; ; spermatid; ; muscle of thigh; ; renal corpuscle; ; fetal liver hematopoietic progenitor cell;
	More reference expression data
	n/a
Gene ontology
Molecular function	catalytic activity; protein homodimerization activity; lyase activity; 4-hydroxy-2-oxoglutarate aldolase activity; protein binding;
Cellular component	mitochondrion; mitochondrial matrix;
Biological process	metabolism; pyruvate biosynthetic process; 4-hydroxyproline catabolic process; glyoxylate catabolic process; oxalate metabolic process; glyoxylate metabolic process;
	Sources:Amigo / QuickGO
Orthologs
	112817
	67432
	ENSG00000241935
	ENSMUSG00000025176
	Q86XE5
	Q9DCU9
	NM_138413; NM_001134670
	NM_026152
	NP_001128142; NP_612422
	NP_080428
	Wikidata
View/Edit Human	View/Edit Mouse

4-Hydroxy-2-oxoglutarate aldolase, mitochondrial (HOGA1) also known as dihydrodipicolinate synthase-like (DHDPSL) is an enzyme that in humans is encoded by the HOGA1 gene. The protein is one of the enzymes (4-hydroxy-2-oxoglutarate aldolase) involved in metabolism of hydroxyproline to glyoxylate. The enzyme overactivity can form excessive glyoxylate from hydroxyproline. Glyoxylate is catabolised to oxalate, resulting in excess excretion of oxalate in urine, predisposing to oxalate stone; a condition known as primary hyperoxaluria type III.