HOGA1

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]

PDBOrtholog search: PDBe RCSB

AliasesHOGA1, C10orf65, DHDPS2, DHDPSL, HP3, NPL2, 4-hydroxy-2-oxoglutarate aldolase 1

External IDsOMIM: 613597; MGI: 1914682; HomoloGene: 12130; GeneCards: HOGA1; OMA:HOGA1 - orthologs

Chr.Chromosome 10 (human)^[1]

Quick facts Available structures, PDB ...

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)[1] Band 10q24.2 Start 97,584,323 bp[1] End 97,612,802 bp[1]
Gene location (Mouse) Chr. Chromosome 19 (mouse)[2] Band 19|19 C3 Start 42,034,049 bp[2] End 42,059,392 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) 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 BioGPS 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 Species Human Mouse Entrez 112817 67432 Ensembl ENSG00000241935 ENSMUSG00000025176 UniProt Q86XE5 Q9DCU9 RefSeq (mRNA) NM_138413 NM_001134670 NM_026152 RefSeq (protein) NP_001128142 NP_612422 NP_080428 Location (UCSC) Chr 10: 97.58 – 97.61 Mb Chr 19: 42.03 – 42.06 Mb PubMed search [3] [4]
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