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
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; ; kidney; ; renal medulla; ; left ventricle; ; body of pancreas; ; Left adrenal gland; ; popliteal artery; ; thoracic aorta; ; ascending aorta; ; gastrocnemius muscle;
	Top expressed in
	proximal tubule; ; liver; ; left lobe of liver; ; kidney; ; yolk sac; ; spermatid; ; renal corpuscle; ; ureter; ; medullary collecting duct; ; spermatocyte;
	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

Last updated October 18, 2022

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.^[5]

Related Research Articles

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Serine—pyruvate aminotransferase is an enzyme that in humans is encoded by the AGXT gene.

Procollagen-lysine,2-oxoglutarate 5-dioxygenase 3 is an enzyme that in humans is encoded by the PLOD3 gene.

Glyoxylate reductase/hydroxypyruvate reductase is an enzyme that in humans is encoded by the GRHPR gene.

M-phase phosphoprotein 6 is an enzyme that in humans is encoded by the MPHOSPH6 gene.

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial is an enzyme that in humans is encoded by the ALDH4A1 gene.

Prolyl 4-hydroxylase subunit alpha-2 is an enzyme that in humans is encoded by the P4HA2 gene.

Primary hyperoxaluria is a rare condition, resulting in increased excretion of oxalate, with oxalate stones being common.

Phosphatidate phosphatase PPAPDC1A also known as phosphatidic acid phosphatase type 2 domain containing 1A is an enzyme that in humans is encoded by the PPAPDC1A gene. PPAPDC1A has phosphatidate phosphatase activity.

3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) is an enzyme in humans that is encoded by the HMGCS2 gene.

COQ3, also known as ubiquinone biosynthesis O-methyltransferase, mitochondrial is an enzyme that in humans is encoded by the COQ3 gene.

Inositol monophosphatase 3 also known as inositol monophosphatase domain-containing protein 1 (IMPAD1) is an enzyme that in humans is encoded by the IMPAD1 gene.

Phosphatidylinositol-5-phosphate 4-kinase, type II, gamma is an enzyme in humans that is encoded by the PIP4K2C gene. It is one of the phosphatidylinositol 4-phosphate 5-kinases.

The human AGXT2 gene encodes the protein Alanine—glyoxylate aminotransferase 2.

Hydroxyacid oxidase 1 is a protein that in humans is encoded by the HAO1 gene.

Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial, also known as alpha-aminoadipate aminotransferase and kynurenine aminotransferase 2, is a mitochondrial enzyme that in humans is encoded by the AADAT gene. It converts alpha-aminoadipate to alpha-ketoadipate. It is also one of the Kynurenine—oxoglutarate transaminases.

Protein geranylgeranyltransferase type I subunit beta is a protein that in humans is encoded by the PGGT1B gene.

Aldo-keto reductase family 1 member C2, also known as bile acid binding protein, 3α-hydroxysteroid dehydrogenase type 3, and dihydrodiol dehydrogenase type 2, is an enzyme that in humans is encoded by the AKR1C2 gene.

Kynurenine aminotransferase 3 is an enzyme that in humans is encoded by the KYAT3 gene. It is one of the Kynurenine—oxoglutarate transaminases.

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000241935 - Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000025176 - Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ Belostotsky R, Seboun E, Idelson GH, Milliner DS, Becker-Cohen R, Rinat C, Monico CG, Feinstein S, Ben-Shalom E, Magen D, Weissman I, Charon C, Frishberg Y (September 2010). "Mutations in DHDPSL are responsible for primary hyperoxaluria type III". American Journal of Human Genetics. 87 (3): 392–9. doi:10.1016/j.ajhg.2010.07.023. PMC 2933339 . PMID 20797690.

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[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000241935 - Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000025176 - Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid20797690-5] Belostotsky R, Seboun E, Idelson GH, Milliner DS, Becker-Cohen R, Rinat C, Monico CG, Feinstein S, Ben-Shalom E, Magen D, Weissman I, Charon C, Frishberg Y (September 2010). "Mutations in DHDPSL are responsible for primary hyperoxaluria type III". American Journal of Human Genetics. 87 (3): 392–9. doi:10.1016/j.ajhg.2010.07.023. PMC 2933339 . PMID 20797690.

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