Anti-PYROXD1 Antibody Picoband® fluoro647 Vervoeging

Pyridine nucleotide-disulphide oxidoreductase domain 1 is a protein that in humans is encoded by the PYROXD1 gene. It is mapped to 12p12.1. This gene encodes a nuclear-cytoplasmic pyridine nucleotide-disulphide reductase (PNDR) . PNDRs are flavoproteins that catalyze the pyridine nucleotide-dependent reduction of thiol residues in other proteins. The encoded protein belongs to the class I pyridine nucleotide-disulphide oxidoreductase family but lacks the C-terminal dimerization domain found in other family members and instead has a C-terminal nitrile reductase domain. It localizes to the nucleus and to striated sarcomeric compartments. Naturally occurring mutations in this gene cause early-onset myopathy with internalized nuclei and myofibrillar disorganization. A pseudogene of this gene has been defined on chromosome 11.

Pyridine nucleotide-disulfide oxidoreductase domain-containing protein 1; PYROXD1

PYROXD1

79912

Q8WU10

Human, Mouse, Rat

No cross-reactivity with other proteins.

E.coli-derived human PYROXD1 recombinant protein (Position: A26-N453) .

Detected in liver, skeletal muscle, kidney, pancreas, spleen, thyroid, testis, ovary, small intestine and colon.

Flow Cytometry

Cell Biology

Immunogen affinity purified.

Liquid

Involved in cellular response to oxidative stress.

1. O'Grady, G. L., Best, H. A., Sztal, T. E., Schartner, V., Sanjuan-Vazquez, M., Donkervoort, S., Neto, O. A., Sutton, R. B., Ilkovski, B., Romero, N. B., Stojkovic, T., Dastgir, J., and 30 others. Variants in the oxidoreductase PYROXD1 cause early-onset myopathy with internalized nuclei and myofibrillar disorganization. Am. J. Hum. Genet. 99: 1086-1105, 2016. 2. Zhang, J.-L., Zhao, W.-G., Wu, K.-L., Wang, K., Zhang, X., Gu, C.-F., Li, Y., Zhu, Y., Wu, J.-G. Human hepatitis B virus X protein promotes cell proliferation and inhibits cell apoptosis through interacting with a serine protease Hepsin. Arch. Virol. 150: 721-741, 2005.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

24145 MW

6

Pyridine nucleotide-disulphide oxidoreductase domain 1

Nucleus. Sarcomere.

Transmembrane protein 240

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.