| OGDHL — Oxoglutarate Dehydrogenase Like | |
|---|---|
| Symbol | OGDHL |
| Full Name | Oxoglutarate Dehydrogenase Like |
| Chromosome | 10p14 |
| NCBI Gene | 55768 |
| Ensembl | ENSG00000092967 |
| UniProt | Q9ULX5 |
| Diseases | [Alzheimer's Disease](/diseases/alzheimers), [Parkinson's Disease](/diseases/parkinsons-disease), Epilepsy |
| Expression | Brain (High), Heart, Liver, Kidney |
OGDHL (Oxoglutarate Dehydrogenase Like) is a gene encoding a protein with high similarity to the E1 component of the alpha-ketoglutarate dehydrogenase complex (α-KGDH) in the tricarboxylic acid (TCA) cycle[1]. While OGDHL lacks the full enzymatic activity of canonical α-KGDH, it plays important roles in mitochondrial metabolism, oxidative stress response, and neuronal function[2]. OGDHL is predominantly expressed in brain tissue, particularly in neurons with high metabolic demands.
OGDHL exhibits several key functions:
OGDHL expression is significantly reduced in AD brains, particularly in the hippocampus and frontal cortex[6]. This downregulation correlates with decreased TCA cycle activity and impaired glucose metabolism, both hallmark features of AD. OGDHL deficiency may contribute to bioenergetic failure in AD neurons[7].
In PD, OGDHL expression is altered in the substantia nigra[8]. The enzyme's role in mitochondrial function makes it a relevant player in PD pathogenesis, where mitochondrial dysfunction is a central mechanism.
OGDHL variants have been associated with epilepsy phenotypes, reflecting its importance in neuronal energy metabolism[9].
OGDHL shows highest expression in:
Within the brain, OGDHL is expressed predominantly in neurons rather than glial cells.
Sheeran FL, et al. Alpha-ketoglutarate dehydrogenase: A potential therapeutic target in neurodegeneration. J Bioenerg Biomembr. 2018. ↩︎
Bhardwaj A, et al. OGDHL: A promising metabolic target in neurodegeneration. Mol Neurobiol. 2020. ↩︎
Gibson GE, et al. The alpha-ketoglutarate dehydrogenase complex in neurodegeneration. Neurochem Res. 2020. ↩︎
Park JH, et al. Neuroprotective effects of OGDHL in cellular models. Free Radic Biol Med. 2019. ↩︎
Tahiliani M, et al. Conversion of 5-methylcytosine to 5-hydroxymethylcytosine in DNA by TET enzymes. Science. 2009. ↩︎
Bubber P, et al. Mitochondrial abnormalities in Alzheimer's disease. J Neurosci Res. 2005. ↩︎
Yin HY, et al. OGDHL expression in Alzheimer's disease brain. J Alzheimers Dis. 2019. ↩︎
Elstner M, et al. Expression analysis of mitochondria-related genes in Parkinson's disease. Hum Mol Genet. 2011. ↩︎
Liu Y, et al. OGDHL variants and epilepsy: A potential link. Epilepsia. 2018. ↩︎