HOGA1 - 4-hydroxy-2-oxoglutarate aldolase 1 Gene

Also Known as HP3; NPL2; DHDPS2; DHDPSL; C10orf65

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 112817

About HOGA1

Cytogenetic location: 10q24.2 Genomic coordinates (GRCh38): 10:97,584,389-97,612,802 (from NCBI)

This gene has 4 transcripts (splice variants), 178 orthologues, 1 paralogue and is associated with 2 phenotypes. Biased expression in kidney (RPKM 27.4), liver (RPKM 9.9) and 5 other tissues.

Summary

The authors of PMID:20797690 cloned this gene while searching for genes in a region of chromosome 10 linked to primary hyperoxalurea type III. They noted that even though the encoded protein has been described as a mitochondrial dihydrodipicolinate synthase-like enzyme, it shares little homology with E. coli dihydrodipicolinate synthase (Dhdps), particularly in the putative substrate-binding region. Moreover, neither lysine biosynthesis nor sialic acid metabolism, for which Dhdps is responsible, occurs in vertebrate mitochondria. They propose that this gene encodes mitochondrial 4-hydroxyl-2-oxoglutarate aldolase (EC 4.1.3.16), which catalyzes the final step in the metabolic pathway of hydroxyproline, releasing glyoxylate and pyruvate. This gene is predominantly expressed in the liver and kidney, and mutations in this gene are found in patients with primary hyperoxalurea type III. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. [provided by RefSeq, Nov 2010]

HOGA1 Products (2)

mRNA	Protein	Name
NM_001134670.2	NP_001128142.1	4-hydroxy-2-oxoglutarate aldolase, mitochondrial isoform 2
NM_138413.4	NP_612422.2	4-hydroxy-2-oxoglutarate aldolase, mitochondrial isoform 1

Gene Ontology

Molecular Function
Biological Process

Molecular Function GO Annotation	Evidence	References	Source
enables (R,S)-4-hydroxy-2-oxoglutarate aldolase activity	IDA IDA: Inferred from direct assay	21998747	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	27499296	GOA
enables protein homodimerization activity	IDA IDA: Inferred from direct assay	21998747	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in 4-hydroxyproline catabolic process	IDA IDA: Inferred from direct assay	21998747	GOA
involved in glyoxylate catabolic process	IMP IMP: Inferred from mutant phenotype	20797690	GOA
involved in glyoxylate metabolic process	IDA IDA: Inferred from direct assay	21998747	GOA
involved in oxalate metabolic process	IMP IMP: Inferred from mutant phenotype	21896830	GOA
involved in pyruvate biosynthetic process	IDA IDA: Inferred from direct assay	21998747	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

HOGA1 Protein Structure

DHDPS

0
100
200
300
327 a.a.

Protein Preferred Names	Protein Names
4-hydroxy-2-oxoglutarate aldolase, mitochondrial	DHDPS-like protein

HOGA1 Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	HOGA1	Q86XE5	CIMAP1A	Homo sapiens	Q96PU9	Validated Y2H	32296183
Intra	HOGA1	Q86XE5	USP47	Homo sapiens	Q96K76	Anti Tag CoIP	33961781
Intra	HOGA1	Q86XE5	USP47	Homo sapiens	Q96K76	Anti Tag CoIP	28514442
Intra	HOGA1	Q86XE5	STARD7	Homo sapiens	Q9NQZ5	Anti Tag CoIP	27499296

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases	Alias
Hyperoxaluria, Primary, Type Iii	Primary Hyperoxaluria Type 3 HP3 Ph Iii Primary Hyperoxaluria Type Iii Hyperoxaluria Primary 3 Hyperoxaluria Non-Hp1/Non-Hp2 Hyperoxaluria Non-Ph I/Ph Ii Form Hyperoxaluria Primary Type Iii
Urolithiasis
Urinary Tract Infection	Urinary Tract Infections Uti Urinary Tract Infection Nos Uti - [Urinary Tract Infection] Uti Nos - [Urinary Tract Infection Nos] Urosepsis Nos E Coli Uti E Coli Urinary Tract Infection Escherichia Coli Uti
Hyperoxaluria, Primary, Type Ii	Primary Hyperoxaluria Type 2 D-Glycerate Dehydrogenase Deficiency Glyoxylate Reductase/Hydroxypyruvate Reductase Deficiency HP2 Oxalosis Ii Glyceric Aciduria L-Glyceric Aciduria Primary Hyperoxaluria, Type Ii Oxalosis 2 Hyperoxaluria Primary 2 Hyperoxaluria Primary Type Ii Ph2 Primary Hyperoxaluria Type Ii
Adenine Phosphoribosyltransferase Deficiency	Aprt Deficiency 2,8-Dihydroxyadenine Urolithiasis APRTD 2,8-Dihydroxyadeninuria Dihydroxyadeninuria Urolithiasis, 2,8-Dihydroxyadenine Urolithiasis, Dha Nephrolithiasis, Dha Dha Crystalline Nephropathy Nephrolithiasis Dha Urolithiasis Dha
Urethral Calculus	Calculus In Urethra Urethral Stone Urethrolithiasis Urethra Calculi Impaction Urethra Calculus Impaction Urethra Stone Calculous Urethritis Urethral Calculi Impaction Urethral Calculus Impaction
Primary Hyperoxaluria	Hyperoxaluria Hyperoxaluria, Primary Oxalosis Primary Oxalosis Congenital Oxaluria D-Glycerate Dehydrogenase Deficiency Glyceric Aciduria Glycolic Aciduria Hepatic Agt Deficiency Oxaluria, Primary Peroxisomal Alanine:Glyoxylate Aminotransferase Deficiency Primary Oxaluria Hyperoxaluria Primary Primary Hyperoxaluria Type 2 Primary Hyperoxaluria, Type I
Xanthinuria	Xanthine Dehydrogenase Deficiency Xanthine Oxidase Deficiency Hereditary Xanthinuria Xanthic Urolithiasis Xanthine Stone Disease Xanthinuria, Type I Combined Deficiency Of Xanthine Dehydrogenase And Aldehyde Oxidase Xdh Deficiency Classic Xanthinuria Xanthinuria, Type Ii Classical Xanthinuria Xanthine Calculus
Purine-Pyrimidine Metabolic Disorder	Inborn Errors Of Purine-Pyrimidine Metabolism Disorder Of Purine Or Pyrimidine Metabolism
Inflammatory Bowel Disease 3	IBD3
Dent Disease 1	Dent Disease Dent'S Disease Dent Disease 2 Dent Disease Type 1 DENT1 Urolithiasis, Hypercalciuric, X-Linked Nephrolithiasis 2 Nphl2 Dent Syndrome Dents Disease Low-Molecular-Weight Proteinuria With Hypercalciuria And Nephrocalcinosis Renal Fanconi Syndrome With Nephrocalcinosis And Renal Stones X-Linked Recessive Hypercalciuric Hypophosphatemic Rickets X-Linked Recessive Nephrolithiasis Fanconi Syndrome, Renal, With Nephrocalcinosis And Renal Stones Nephrolithiasis, Hypercalciuric, X-Linked Nephrolithiasis-Hypercalciuria X-Linked Recessive Nephrolithiasis, X-Linked Recessive Dent Disease, Type 1
Hypophosphatemic Rickets, X-Linked Recessive	Hypophosphatemic Rickets X-Linked Recessive Hypophosphatemic Rickets XLRHR Hypophosphatemic Rickets Disorders Rickets Hypophosphatemic Rickets, Hypophosphatemic, X-Linked Recessive Familial Hypophosphatemic Rickets
Cystinuria	CSNU Cystinuria Type B Cystinuria Type A Cystinuria, Type I, Formerly Csnu1, Formerly Cystinuria, Type Ii, Formerly Cystinuria, Type Iii, Formerly Csnu3, Formerly Cystinuria, Type Non-I, Formerly Cystinuria-Lysinuria Cystinuria-Lysinuria Syndrome Csnu1 Csnu3 Cystinuria 1 Cystinuria Type A/B Cystinuria Type I Cystinuria Type Ii Cystinuria Type Iii Cystinuria Type Non-I Cystinuria, Type A/B Cystinuria Type 1 Cystinuria - Lysinuria Csnu - [Cystinuria] Cystine Disease

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (2)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS06282	HOGA1 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS22556	Hoga1 Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Felis catus	HOGA1	VGNC	VGNC:62829
Rattus norvegicus	HOGA1	RGD	RGD:1310475
Mus musculus	HOGA1	MGD	MGI:1914682
Bos taurus	HOGA1	VGNC	VGNC:29900
Canis familiaris	HOGA1	VGNC	VGNC:41736
Others	HOGA1	NCBI