Anti-PUF60 Antibody Picoband®

SKU: A05744-2

Size: 100 μg

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

Form: Lyophilized

Reactivity: Human,Mouse

Applications: WB,IHC,IP,Flow Cytometry,ELISA

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

Gene Name: PUF60

Specificity:

Background: This gene encodes a nucleic acid-binding protein that plays a role in a variety of nuclear processes, including pre-mRNA splicing and transcriptional regulation. The encoded protein forms a complex with the far upstream DNA element (FUSE) and FUSE-binding protein at the myelocytomatosis oncogene (MYC) promoter. This complex represses MYC transcription through the core-TFIIH basal transcription factor. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene.

Immunogen: E.coli-derived human PUF60 recombinant protein (Position: Q48-A559). Human PUF60 shares 98.8% amino acid (aa) sequence identity with both mouse and rat PUF60.

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. Dauber, A., Golzio, C., Guenot, C., Jodelka, F. M., Kibaek, M., Kjaergaard, S., Leheup, B., Martinet, D., Nowaczyk, M. J. M., Rosenfeld, J. A., Zeesman, S., Zunich, J., Beckmann, J. S., Hirschhorn, J. N., Hastings, M. L., Jacquemont, S., Katsanis, N. SCRIB and PUF60 are primary drivers of the multisystemic phenotypes of the 8q24.3 copy-number variant. Am. J. Hum. Genet. 93: 798-811, 2013. Note: Erratum: Am. J. Hum. Genet. 93: 994 only, 2013. 2. El Chehadeh, S., Kerstjens-Frederikse, W. S., Thevenon, J., Kuentz, P., Bruel, A.-L., Thauvin-Robinet, C., Bensignor, C., Dollfus, H., Laugel, V., Riviere, J.-B., Duffourd, Y., Bonnet, C., and 19 others. Dominant variants in the splicing factor PUF60 cause a recognizable syndrome with intellectual disability, heart defects and short stature. Europ. J. Hum. Genet. 25: 43-51, 2017. 3. Graziano, C., Gusson, E., Severi, G., Isidori, F., Wischmeijer, A., Brugnara, M., Seri, M., Rossi, C. A de novo PUF60 mutation in a child with a syndromic form of coloboma and persistent fetal vasculature. Ophthal. Genet. 38: 590-592, 2017.

Uniprot ID: Q9UHX1

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: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 60 kDa

Calculated Molecular Weight: 60 kDa

Gene ID: 22827

Protein Name: Poly

Gene Full Name: poly(U) binding splicing factor 60

Synonyms: FBP interacting repressor, FIR, PUF60, Ro binding protein 1, RoBP1, RoBPI, Siah binding protein 1, Siah BP1, SIAHBP1