Anti-SQRDL/SQOR Antibody Picoband®

SKU: A31817

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,IHC,ICC,IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: SQOR

Specificity:

Background: The protein encoded by this gene may function in mitochondria to catalyze the conversion of sulfide to persulfides, thereby decreasing toxic concencrations of sulfide. Alternative splicing results in multiple transcript variants that encode the same protein.

Immunogen: E.coli-derived human SQRDL/SQOR recombinant protein (Position: R12-E321). Human SQOR shares 86.1% amino acid (aa) sequence identity with mouse SQOR.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Friederich, M. W., Elias, A. F., Kuster, A., Laugwitz, L., Larson, A. A., Landry, A. P., Ellwood-Digel, L., Mirsky, D. M., Dimmock D., Haven, J., Jiang, H., MacLean, K. N., Styren, K., Schoof, J., Goujon, L., Lefrancois, T., Friederich, M., Couglin, C. R., II, Banerjee, R., Haack, T. B., Van Hove, J. L. K. Pathogenic variants in SQOR encoding sulfide:quinone oxidoreductase are a potentially treatable cause of Leigh disease. J. Inherit. Metab. Dis. 43: 1024-1036, 2020. 2. Gonzalez-Garcia, P., Hidalgo-Gutierrez, A., Mascaraque, C., Barriocanal-Casado, E., Bakkali, M., Ziosi, M., Abdihankyzy, U. B., Sanchez-Hernandez, S., Escames, G., Prokish, H., Martin, F., Quinzii, C. M., Lopez, L. C. Coenzyme Q10 modulates sulfide metabolism and links the mitochondrial respiratory chain to pathways associated to one carbon metabolism. Hum. Molec. Genet. 29: 3296-3311, 2020. 3. Hartz, P. A. Personal Communication. Baltimore, Md. 09/01/2017.

Uniprot ID: Q9Y6N5

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross reactivity with other proteins.

Isotype: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 50 kDa

Calculated Molecular Weight: 52588 MW

Gene ID: 58472

Protein Name: Sulfide:quinone oxidoreductase, mitochondrial

Gene Full Name: sulfide quinone oxidoreductase

Synonyms: CGI 44; SQOR; SQRDL