SURF-1 Antibody (H-7): sc-365159
- SURF-1 Antibody (H-7) is a mouse monoclonal IgG1 κ SURF-1 antibody provided at 200 µg/ml
- specific for an epitope mapping between amino acids 127-153 within an internal region of SURF-1 of human origin
- SURF-1 Antibody (H-7) is recommended for detection of SURF-1 of mouse, rat and human origin by WB, IP, IF and ELISA
- Anti-SURF-1 Antibody (H-7) is available conjugated to agarose for IP; HRP for WB, IHC(P) and ELISA; and to either phycoerythrin or FITC for IF, IHC(P) and FCM
- also available conjugated to Alexa Fluor® 488, Alexa Fluor® 546, Alexa Fluor® 594 or Alexa Fluor® 647 for WB (RGB), IF, IHC(P) and FCM, and for use with RGB fluorescent imaging systems, such as iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- also available conjugated to Alexa Fluor® 680 or Alexa Fluor® 790 for WB (NIR), IF and FCM; for use with Near-Infrared (NIR) detection systems, such as LI-COR®Odyssey®, iBright™ FL1000, FluorChem™, Typhoon, Azure and other comparable systems
- m-IgG1 BP-HRP is the preferred secondary detection reagent for SURF-1 Antibody (H-7) for WB applications. This reagent is now offered in a bundle with SURF-1 Antibody (H-7) (see ordering information below).
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SURF-1 Antibody (H-7) is a mouse monoclonal IgG1 kappa light chain antibody that detects SURF-1 protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). SURF-1 (H-7) antibody 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. SURF-1 protein plays a crucial role in the assembly of complex IV (cytochrome c oxidase) of the mitochondrial respiratory chain, which is essential for cellular energy production. Located in the inner mitochondrial membrane, SURF-1 is integral to the proper functioning of this complex, and mutations in SURF-1 gene can lead to severe consequences, such as cytochrome c oxidase complex IV deficiency. This deficiency is associated with Leigh syndrome, a devastating neurological disorder characterized by rapid progression of encephalopathy and necrotic lesions in subcortical brain regions. Patients with Leigh syndrome often experience significant motor deficits and early-onset mortality, highlighting SURF-1′s importance in mitochondrial function and overall cellular health. Additionally, SURF-1 mutations can result in morphological abnormalities in the heart, brain, and skeletal muscle, further underscoring this protein′s critical role in maintaining normal physiological processes.
Alexa Fluor® is a trademark of Molecular Probes Inc., OR., USA
LI-COR® and Odyssey® are registered trademarks of LI-COR Biosciences
SURF-1 Antibody (H-7) References:
- Loss-of-function mutations of SURF-1 are specifically associated with Leigh syndrome with cytochrome c oxidase deficiency. | Tiranti, V., et al. 1999. Ann Neurol. 46: 161-6. PMID: 10443880
- Characterization of SURF-1 expression and Surf-1p function in normal and disease conditions. | Tiranti, V., et al. 1999. Hum Mol Genet. 8: 2533-40. PMID: 10556302
- Differential features of patients with mutations in two COX assembly genes, SURF-1 and SCO2. | Sue, CM., et al. 2000. Ann Neurol. 47: 589-95. PMID: 10805329
- Myc and YY1 mediate activation of the Surf-1 promoter in response to serum growth factors. | Vernon, EG. and Gaston, K. 2000. Biochim Biophys Acta. 1492: 172-9. PMID: 10858544
- Three novel SURF-1 mutations in Japanese patients with Leigh syndrome. | Ogawa, Y., et al. 2002. Pediatr Neurol. 26: 196-200. PMID: 11955926
- MR findings in Leigh syndrome with COX deficiency and SURF-1 mutations. | Farina, L., et al. 2002. AJNR Am J Neuroradiol. 23: 1095-100. PMID: 12169463
- Mutations of SURF-1 in Leigh disease associated with cytochrome c oxidase deficiency. | Tiranti, V., et al. 1998. Am J Hum Genet. 63: 1609-21. PMID: 9837813
- Constitutive knockout of Surf1 is associated with high embryonic lethality, mitochondrial disease and cytochrome c oxidase deficiency in mice. | Agostino, A., et al. 2003. Hum Mol Genet. 12: 399-413. PMID: 12566387
Ordering Information
| Product Name | Catalog # | UNIT | Price | Qty | FAVORITES | |
SURF-1 Antibody (H-7) | sc-365159 | 200 µg/ml | $322.00 | |||
SURF-1 Antibody (H-7): m-IgG1 BP-HRP Bundle | sc-531701 | 200 µg Ab; 20 µg BP | $361.00 | |||
SURF-1 Antibody (H-7) AC | sc-365159 AC | 500 µg/ml, 25% agarose | $424.00 | |||
SURF-1 Antibody (H-7) HRP | sc-365159 HRP | 200 µg/ml | $322.00 | |||
SURF-1 Antibody (H-7) FITC | sc-365159 FITC | 200 µg/ml | $336.00 | |||
SURF-1 Antibody (H-7) PE | sc-365159 PE | 200 µg/ml | $349.00 | |||
SURF-1 Antibody (H-7) Alexa Fluor® 488 | sc-365159 AF488 | 200 µg/ml | $364.00 | |||
SURF-1 Antibody (H-7) Alexa Fluor® 546 | sc-365159 AF546 | 200 µg/ml | $364.00 | |||
SURF-1 Antibody (H-7) Alexa Fluor® 594 | sc-365159 AF594 | 200 µg/ml | $364.00 | |||
SURF-1 Antibody (H-7) Alexa Fluor® 647 | sc-365159 AF647 | 200 µg/ml | $364.00 | |||
SURF-1 Antibody (H-7) Alexa Fluor® 680 | sc-365159 AF680 | 200 µg/ml | $364.00 | |||
SURF-1 Antibody (H-7) Alexa Fluor® 790 | sc-365159 AF790 | 200 µg/ml | $364.00 | |||
SURF-1 (H-7) Neutralizing Peptide | sc-365159 P | 100 µg/0.5 ml | $69.00 |