HOGA1
HOGA1 (4-hydroxy-2-oxoglutarate aldolase 1) is a mitochondrial aldolase that catalyses the final step in the metabolic pathway of hydroxyproline. It is also known as DHDPS2, DHDPSL, NPL2, C10orf65 and FLJ37472. Browse Assay Genie's current range of HOGA1 research reagents below, or read on for the biology behind the target.
HOGA1 is a mitochondrial 4-hydroxy-2-oxoglutarate aldolase that catalyses the final step in the metabolic pathway of hydroxyproline. In this reaction the enzyme releases glyoxylate and pyruvate. It is classed as an oxo-acid lyase (EC 4.1.3.16) and belongs to the DapA family.
The HOGA1 gene is predominantly expressed in the liver and kidney, and the encoded protein localises to the mitochondrion. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene. The mouse ortholog is Hoga1, which is useful for comparative and model-based work.
The gene was cloned by researchers searching a region of chromosome 10 linked to primary hyperoxaluria type III. Earlier it was described as a mitochondrial dihydrodipicolinate synthase-like enzyme, hence the aliases DHDPS2 and DHDPSL and the previous name dihydrodipicolinate synthase-like, mitochondrial. However, the protein shares little homology with E. coli dihydrodipicolinate synthase, particularly in the putative substrate-binding region. Neither lysine biosynthesis nor sialic acid metabolism, the processes for which that bacterial enzyme is responsible, occurs in vertebrate mitochondria. The authors therefore proposed that the gene encodes the mitochondrial 4-hydroxy-2-oxoglutarate aldolase. Mutations in HOGA1 are found in patients with primary hyperoxaluria type III, which makes the protein a focus for studies of hydroxyproline and glyoxylate metabolism.
HOGA1 catalyses the final step of the hydroxyproline pathway, so it is central to studies of how this amino acid is broken down.
Mutations in the HOGA1 gene are found in patients with primary hyperoxaluria type III, making it a key target in this disease.
As a mitochondrial aldolase releasing glyoxylate and pyruvate, HOGA1 is relevant to research on mitochondrial lyases and the DapA family.
HOGA1 is predominantly expressed in the liver and kidney, supporting its use in studies of metabolism in these tissues.
HOGA1 is a mitochondrial 4-hydroxy-2-oxoglutarate aldolase (EC 4.1.3.16). It catalyses the final step in the metabolic pathway of hydroxyproline, releasing glyoxylate and pyruvate. It is predominantly expressed in the liver and kidney.
HOGA1 is also known as DHDPS2, DHDPSL, NPL2, C10orf65 and FLJ37472. Previous names include chromosome 10 open reading frame 65 and dihydrodipicolinate synthase-like, mitochondrial. Alternatively spliced transcript variants encoding different isoforms have been noted.
Mutations in the HOGA1 gene are found in patients with primary hyperoxaluria type III. A mouse ortholog, Hoga1, exists; check the datasheet of any product for its validated reactivity.
Other enzymes of glyoxylate, hydroxyproline and mitochondrial metabolism with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt Q86XE5; product specifications from Assay Genie datasheets.