NUBPL Antibody (G-7): sc-393245

NUBPL Antibody (G-7) is a mouse monoclonal IgG1 antibody that detects NUBPL in human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). NUBPL, a 319 amino acid mitochondrial protein, is a crucial member of the Mrp/NBP35 ATP-binding protein family and exists in two alternatively spliced isoforms. Anti-NUBPL antibody (G-7) plays a vital role in studying the assembly of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I), where NUBPL is believed to facilitate the delivery of iron-sulfur clusters to the complex I subunits, a process essential for proper cellular respiration. NUBPL localization within the mitochondria underscores its importance in energy production, as defects in NUBPL can lead to mitochondrial complex I deficiency (MT-C1D), resulting in a spectrum of severe health issues ranging from lethal neonatal conditions to adult-onset neurodegenerative diseases. NUBPL is predominantly expressed in the liver and kidney, with significant levels also found in the small intestine and brain, highlighting its critical role in various physiological processes. NUBPL dysfunction is associated with a range of phenotypes, including macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and certain forms of Parkinson′s disease, making NUBPL monoclonal antibody (G-7) an invaluable tool for research into mitochondrial disorders and related pathologies.

Alexa Fluor^® is a trademark of Molecular Probes Inc., OR., USA

LI-COR^® and Odyssey^® are registered trademarks of LI-COR Biosciences

NUBPL Antibody (G-7) References:

1. NUBPL mutations in patients with complex I deficiency and a distinct MRI pattern.  |  Kevelam, SH., et al. 2013. Neurology. 80: 1577-83. PMID: 23553477
2. NUBPL, a novel metastasis-related gene, promotes colorectal carcinoma cell motility by inducing epithelial-mesenchymal transition.  |  Wang, Y., et al. 2017. Cancer Sci. 108: 1169-1176. PMID: 28346728
3. Mitochondrial complex I NUBPL mutations cause combined dystonia with bilateral striatal necrosis and cerebellar atrophy.  |  Balint, B., et al. 2019. Eur J Neurol. 26: 1240-1243. PMID: 30897263
4. Novel compound heterozygous pathogenic variants in nucleotide-binding protein like protein (NUBPL) cause leukoencephalopathy with multi-systemic involvement.  |  Protasoni, M., et al. 2020. Mol Genet Metab. 129: 26-34. PMID: 31787496
5. Pathogenic variants in NUBPL result in failure to assemble the matrix arm of complex I and cause a complex leukoencephalopathy with thalamic involvement.  |  Friederich, MW., et al. 2020. Mol Genet Metab. 129: 236-242. PMID: 31917109
6. NUBPL mitochondrial disease: new patients and review of the genetic and clinical spectrum.  |  Kimonis, V., et al. 2021. J Med Genet. 58: 314-325. PMID: 32518176
7. Loss-of-Function NUBPL Mutation May Link Parkinson's Disease to Recessive Complex I Deficiency.  |  Eis, PS., et al. 2020. Front Neurol. 11: 555961. PMID: 33224084
8. Expanding the Spectrum of NUBPL-Related Leukodystrophy.  |  Tonduti, D., et al. 2023. Neuropediatrics. 54: 161-166. PMID: 36868263
9. Murine trophoblast organoids as a model for trophoblast development and CRISPR-Cas9 screening.  |  Mao, Q., et al. 2023. Dev Cell. 58: 2992-3008.e7. PMID: 38056451
10. Disulfidptosis and ferroptosis related genes define the immune microenvironment and NUBPL serves as a potential biomarker for predicting prognosis and immunotherapy response in bladder cancer.  |  Zhang, X., et al. 2024. Heliyon. 10: e37638. PMID: 39290277

The preferred NUBPL antibody is NUBPL Antibody (G-11): sc-398217.