NDUFS3 Recombinant Monoclonal Antibody

Code CSB-RA224121A0HU
Size US$210
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Image
  • Western Blot
    Positive WB detected in: 293 whole cell lysate, HepG2 whole cell lysate,
    All lanes: NDUFS3 antibody at 1:2000
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    Predicted band size: 31, 15 KDa
    Observed band size: 31 kDa
  • IHC image of CSB-RA224121A0HU diluted at 1:100 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
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Product Details

Uniprot No.
Target Names
NDUFS3
Alternative Names
NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial (EC 1.6.5.3) (EC 1.6.99.3) (Complex I-30kD) (CI-30kD) (NADH-ubiquinone oxidoreductase 30 kDa subunit), NDUFS3
Species Reactivity
Human
Immunogen
A synthesized peptide derived from human NDUFS3
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Clonality
Monoclonal
Isotype
Rabbit IgG
Clone No.
1H12
Purification Method
Affinity-chromatography
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Form
Liquid
Tested Applications
ELISA, WB, IHC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Description

NDUFS3 is a subunit of the NADH: ubiquinone oxidoreductase ETC complex I, which catalyzes the initial stage of electron transfer from NADH to a noncovalently bound flavin mononucleotide and subsequently to the terminal acceptor ubiquinone via a succession of iron-sulfur clusters. NDUFS3 is recruited to the inner mitochondrial membrane to form an early assembly intermediate with NDUFS2 and plays a critical role in the correct assembly of complex I. It starts the complex I assembly process in the mitochondrial matrix. Complex I function is disrupted by NDUFS3 cleavage, resulting in superoxide-dependent but MOMP-independent cell death. NDUFS3 gene missense mutations cause severe encephalomyopathy, including Leigh syndrome (LS).

CUSABIO designed the vector clones for the expression of a recombinant NDUFS3 antibody in mammalian cells. The vector clones were obtained by inserting the NDUFS3 antibody heavy and light chains into the plasma vectors. The recombinant NDUFS3 antibody was purified from the culture medium through Affinity-chromatography. It can be used to detect NDUFS3 protein from Human in the ELISA, WB, IHC.

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Target Background

Function
Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor. Essential for the catalytic activity and assembly of complex I.
Gene References into Functions
  1. identified a novel Leigh syndrome causing NDUFS3 mutation and expanded the clinical spectrum caused by NDUFS3 mutations in this study. PMID: 30140060
  2. The results suggest that the impaired mitochondrial activity could be due to the broken interaction between DJ-1 and NDUFS3 and that downregulation of DJ-1 in sperm and testes contributes to AS pathogenesis. PMID: 29849492
  3. The NDUFS3 expressed in the temporal cortex in patient with late-onset Alzheimer's disease. PMID: 28242297
  4. High NDUFS3 expression is associated with IgA nephropathy. PMID: 25770168
  5. pH stability of w-t and Leigh syndrome mutant varied at extreme acidic pH. the molten globule like structure of w-t at pH1 was absent in case of the mutant protein. Both the w-t and mutant proteins showed multi-step thermal and Gdn-HCl induced unfolding PMID: 24028823
  6. NDUFS3 is down-regulated in serous ovarian adenocarcinoma PMID: 23446378
  7. Expressions of GRIM-19, NDUFS3, and extracellular matrix elements are correlated with invasive capabilities of breast cancer cell lines. PMID: 23630608
  8. a novel, biomarker potential of a mitochondrial complex I subunit protein, NDUFS3 - as a robust indicator of breast cancer progression and invasiveness as well as of hypoxia/necrosis in clinical specimens of invasive ductal carcinoma, was uncovered. PMID: 21867691
  9. Decreased protein levels of complex I 30-kDa subunit in fetal Down syndrome brains. PMID: 11771736

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Subcellular Location
Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
Protein Families
Complex I 30 kDa subunit family
Database Links

HGNC: 7710

OMIM: 603846

KEGG: hsa:4722

STRING: 9606.ENSP00000263774

UniGene: Hs.502528

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