OLA1{Obg-like ATPase 1}rabbit.pAb
- Known as:
- OLA1{Obg-like ATPase 1}host: rabbit.pAb
- Catalog number:
- 201-20-3949
- Product Quantity:
- 0.2ml
- Category:
- -
- Supplier:
- Shanghai Sunred
- Gene target:
- OLA1{Obg-like ATPase 1}rabbit.pAb
Ask about this productRelated genes to: OLA1{Obg-like ATPase 1}rabbit.pAb
- Gene:
- MT-ATP6 NIH gene
- Name:
- mitochondrially encoded ATP synthase membrane subunit 6
- Previous symbol:
- MTATP6, RP
- Synonyms:
- ATP6, ATPase-6, Su6m
- Chromosome:
- mitochondria
- Locus Type:
- gene with protein product
- Date approved:
- 1989-10-12
- Date modifiied:
- 2017-11-22
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- - Source: PubMed
Publication date: 2026/04/25
Xiao ChangruiZhu DavidPryor JonFrutiger Sally AClark H BrentCasey Hannah LBower MatthewYu Guo-YunDu XiaofeiToro CamiloGomez Christopher M - Circular RNAs (circRNAs) are covalently closed, single-stranded RNA molecules generated from both nuclear and mitochondrial genomes. Several nuclear-encoded circRNAs have been reported to be conserved in sequence and function across animals. However, the evolutionary conservation and physiological significance of mitochondria-encoded circRNAs (mecciRNAs) remain largely unexplored. Here, by analyzing mitochondrial RNA sequencing data from human and mouse cells, we identify a conserved mecciRNA derived from the locus, termed mecciATP6. We show that mecciATP6 modulates mitochondrial homeostasis by binding and regulating the protein abundance of an evolutionarily conserved RNA-binding protein (RBP) HNRNPA3. Our data provide a conceptual framework for defining mecciRNA conservation across mammals and uncover a conserved mecciRNA-protein regulatory mechanism linked to mitochondrial homeostasis. - Source: PubMed
Publication date: 2026/03/19
Cao XiaoyangSui JuzhengShan GeLiu Xu - Primary mitochondrial disorders are clinically and genetically heterogeneous and remain underdiagnosed in resource-limited settings. We performed a retrospective observational study (March 2016- January 2024) at a tertiary neurology center in Eastern India to characterize the clinical, biochemical, neuroimaging, electrophysiological, and molecular features of suspected mitochondrial disease and to explore interpretable machine-learning approaches for syndromic stratification. Forty-eight patients from 42 unrelated families were classified as mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS; n = 17), chronic progressive external ophthalmoplegia (CPEO; n = 14), Leber hereditary optic neuropathy (LHON; n = 10), or Leigh syndrome (n = 7). Mean age at presentation was 23.9 years (range: 9 months-60 years), with a slight male predominance. Neuroimaging was abnormal in 23/48 (47.9%) and showed syndrome-concordant patterns, including stroke-like cortical lesions in MELAS and symmetric basal ganglia involvement in Leigh syndrome; brain magnetic resonance imaging was typically normal in CPEO. Elevated blood and/or cerebrospinal fluid lactate was common, and electroencephalographic abnormalities were concentrated in MELAS and Leigh syndrome. Targeted molecular testing in a subset identified pathogenic mtDNA variants consistent with phenotype, including MT-TL1 variants in MELAS, m.11778G>A in MT-ND4 in LHON, and m.8993T>G in MT-ATP6 in Leigh syndrome; no mtDNA deletions were detected in tested CPEO cases. Decision tree and random forest models highlighted clinically intuitive discriminators (e.g., visual loss, external ophthalmoplegia/ptosis, and seizure phenotype), supporting their potential role as transparent triage tools for targeted molecular evaluation. This cohort provides the first detailed characterization of mitochondrial syndromes in Eastern India and supports a pragmatic diagnostic framework integrating bedside phenotyping, targeted assays, and interpretable machine learning. - Source: PubMed
Publication date: 2026/03/16
Banerjee SubhadeepMondal RitwickDeb ShramanaShome GouravChakraborty SharanyaSaha ChinmayPandit AlakSen GargiBhattacharya SreelaSen PurbitaBhattacharya Nitai PRoy JayantaChowdhury AnjanBenito-León Julián - In recent years, an increasing number of individuals have traveled to or resided in plateau regions for various reasons. The hypobaric hypoxia characteristics of plateau environments represents a key risk factor for acute mountain sickness (AMS). The pathogenesis of AMS remains incompletely understood. The present study aimed to explore the association between the A8923G point mutation in the mitochondrial MT-ATP6 gene and AMS. - Source: PubMed
Publication date: 2026/02/26
Zhang MingleiLi RuizheLiu YiningLi Zongbin - This report describes the potential diagnostic value of decreased plasma citrulline (pCit) levels for the early recognition of --related mitochondrial disease. Two cases were reported, and relevant literature was reviewed. Case 1: Onset occurred at 3 months of age with an acute presentation that rapidly progressed to metabolic crisis, multiorgan failure, and central respiratory failure, resulting in death in early infancy. Case 2: Onset occurred at 6 months of age with progressive developmental delay. Brain magnetic resonance imaging revealed bilateral symmetric basal ganglia lesions, and Leigh syndrome was diagnosed. Following citrulline supplementation and comprehensive intervention, improvements were observed in intellectual development and metabolic indices. Both patients carried the - variant m.8993T>G (p.L156R), confirming --associated mitochondrial disease. This case series indicates that decreased pCit on newborn screening is an early biochemical marker of --associated mitochondrial disease. Early diagnosis and metabolic intervention are beneficial for prognosis. - Source: PubMed
Yan Hui-MingQuan YingZhou YingJiang LuoZhang Liang-YuWan Zheng-QingXi Hui