NUBPL Antibody (G-11): sc-398217

NUBPL Antibody (G-11) is a mouse monoclonal IgG2a kappa light chain antibody that detects NUBPL protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). anti-NUBPL antibody (G-11) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. NUBPL (nucleotide-binding protein-like) is a crucial 319 amino acid mitochondrial protein that plays a vital role in the assembly of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). NUBPL delivers iron-sulfur clusters to complex I subunits, critical for proper functioning of the respiratory chain and energy production in cells. NUBPL expression is highest in liver and kidney, with significant levels in small intestine and brain, highlighting importance in various metabolic processes. Defects in NUBPL are associated with mitochondrial complex I deficiency (MT-C1D), a serious condition leading to disorders ranging from lethal neonatal diseases to adult-onset neurodegenerative conditions. MT-C1D can manifest as macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and certain forms of Parkinson′s disease, underscoring NUBPL′s critical role in mitochondrial health and overall cellular function.

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NUBPL Antibody (G-11) References:

1. Human mitochondrial complex I in health and disease.  |  Smeitink, J. and van den Heuvel, L. 1999. Am J Hum Genet. 64: 1505-10. PMID: 10330338
2. Respiratory chain complex I deficiency.  |  Triepels, RH., et al. 2001. Am J Med Genet. 106: 37-45. PMID: 11579423
3. Initiation of neuronal damage by complex I deficiency and oxidative stress in Parkinson's disease.  |  Tretter, L., et al. 2004. Neurochem Res. 29: 569-77. PMID: 15038604
4. Leber's hereditary optic neuropathy and complex I deficiency in muscle.  |  Larsson, NG., et al. 1991. Ann Neurol. 30: 701-8. PMID: 1763894
5. Human ind1, an iron-sulfur cluster assembly factor for respiratory complex I.  |  Sheftel, AD., et al. 2009. Mol Cell Biol. 29: 6059-73. PMID: 19752196
6. High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency.  |  Calvo, SE., et al. 2010. Nat Genet. 42: 851-8. PMID: 20818383
7. Next-generation sequencing in molecular diagnosis: NUBPL mutations highlight the challenges of variant detection and interpretation.  |  Tucker, EJ., et al. 2012. Hum Mutat. 33: 411-8. PMID: 22072591
8. Massive and exclusive pontocerebellar damage in mitochondrial disease and NUBPL mutations.  |  Tenisch, EV., et al. 2012. Neurology. 79: 391. PMID: 22826544
9. Leukodystrophy Associated with Mitochondrial Complex 1 Deficiency Due to Mutation in NUBPL Gene-An Unusual Follow-Up Finding.  |  Peter S, B. and Vandana G, S. 2023. Indian J Radiol Imaging. 33: 132-135. PMID: 36855717
10. RNA sequencing-based approaches to identifying disulfidptosis-related diagnostic clusters and immune landscapes in osteoporosis.  |  Zhang, P., et al. 2024. Aging (Albany NY). 16: 8198-8216. PMID: 38738994
11. Classification and functional analysis of disulfidptosis-associated genes in sepsis.  |  He, S., et al. 2024. J Cell Mol Med. 28: e70020. PMID: 39400961
12. Human complex I deficiency: clinical spectrum and involvement of oxygen free radicals in the pathogenicity of the defect.  |  Robinson, BH. 1998. Biochim Biophys Acta. 1364: 271-86. PMID: 9593934

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-NUBPL Antibody (G-11) has 0 citations in a variety of scientific publications.

Primary antibodies like Anti-NUBPL Antibody (G-11) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

NUBPL Antibody (G-11) Specifications

Alternate Product Name: NUBPL

Product Clone Name: G-11

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2a kappa light chain

Amino Acids: 46-69

Epitope Target: NUBPL

Epitope Species: human

Product Reactivity/Detection: NUBPL

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: equine, canine, bovine and avian

Additional Non-Mammalian Reactivity: avian

Applications: WB, IP, IF, ELISA

Vialing Information: Each vial contains 200 µg IgG_2a kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 0 citations in various scientific publications

Available Conjugates/Forms for NUBPL Antibody (G-11): AC: Agarose, AF488: Alexa Fluor^® 488, AF546: Alexa Fluor^® 546, AF594: Alexa Fluor^® 594, AF647: Alexa Fluor^® 647, AF680: Alexa Fluor^® 680, AF790: Alexa Fluor^® 790, FITC: Fluoroscein, HRP: Horseradish Peroxidase, P: Blocking Peptide, PE: Phycoerythrin